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Radiotherapy in the management of solitary extramedullary plasmacytoma
Abstract
Solitary extramedullary plasmacytoma (SEP) is a rare tumour for which the standard treatment remains local radiotherapy (RT). We present a study of a multi-institutional experience, between 1980 and 1999, in an attempt to better understand the natural history of SEP and to identify possible prognostic factors.
The records at Radiation Oncology Victoria and Peter MacCallum Cancer Centre, Melbourne, were used for the identification of patients. The inclusion criteria were as follows: (i) histological confirmation of clonal plasma cells involving a single extramedullary site with or without lymph node involvement; (ii) no histological evidence of bone marrow involvement; (iii) no evidence of distant bone lesion on radiographic skeletal survey (bone erosions adjacent to the primary thought to be due to contiguous involvement were permitted) and (iv) no anaemia, hypercalcaemia or renal impairment due to plasma cell dyscrasia.
A total of 16 patients was identified, with a median follow up of 66 months (range 12-211 months). The head and neck region accounted for the majority of presentations (88%). Among all patients, a serum mono-clonal paraprotein was found in three patients and bone erosion was identified in seven patients. All patients received local RT, although two patients also received elective nodal irradiation (ENI). The median RT dose was 45 Gy (range 40-50.4 Gy). Local control was achieved in all patients (100%), however, regional recurrence outside the RT fields occurred in 2/16. Multiple myeloma (MM) developed in five patients, all within 5 years. The predicted 10-year myeloma free survival is 75% and 10-year overall survival is 54%.
RT can achieve excellent local control of SEP. The rate of conversion to MM is 31%. Moderate dose RT of at least 40 Gy using limited radiation fields is recommended, although ENI should be considered if toxicity is not increased.
... EMPs are often discrete isolated masses that affect the upper aerodigestive tract (>80% of EMPs), but may rarely involve other sites, including the kidneys. [1][2][3][4][5][6][7] Due to the prevalence of multiple myeloma and the rarity of EMP diagnoses, it is essential to exclude systemic coexistence or other mimicking diseases as the causative reason for a plasmacytoma. [8][9][10] Plasma cell neoplasms accounted for w23% of all non-Hodgkin lymphoma malignancies in the United States in 2016, with >90% attributed to multiple myeloma, and <5%, to extraosseous plasmacytomas. ...
... 13 Given the rarity of EMPs, there are limited recommendations and guidelines for such malignancies. 1,[4][5][6]8,[12][13][14][15][16] Case Report In 2012, a 69-year-old white man was referred to our clinic for evaluation of severely decreased kidney function and a history of chronic back pain with right-sided flank pain. Initial assessment was notable for elevated creatinine levels, which increased to a peak of 4.6 mg/dL (estimated glomerular filtration rate of 12 mL/min/1.73 ...
... 16 Three general approaches are reported: surgery, chemotherapy, and radiotherapy. 1,[3][4][5][6]8,[12][13][14]17 Treatment of EMPs varies from that of myeloma because bone marrow is also involved. Given bilateral involvement in our patient, we pursued a chemotherapy solution instead of radiotherapy. ...
Plasmacytomas are rare immunoproliferative monoclonal plasma cell diseases of lymphoid lineage that may present in an isolated or systemic manner. Systemic involvement is much more common than occurrences isolated to a particular organ, and for this reason, it is imperative to rule out systemic involvement for osseous and nonosseous isolated neoplasms. These neoplasms present unique challenges due to their location, extent of involvement, vague presentation, and dearth of treatment protocol. We report the case of a 69-year-old man who developed chronic kidney disease stage 4 between 2009 and 2012. Precipitous kidney failure, anorexia, fatigue, and flank pain necessitated clinical follow-up that ultimately led to thorough imaging and bilateral kidney biopsy. Protein electrophoresis, immunohistochemistry, and immunofluorescence were all consistent with bilateral renal extramedullary plasmacytomas. Treatment recommendations are often limited to prior case successes; however, chemotherapy, radiation, and surgery are the mainstay of treatment. Although surgery or combined therapy provides the best results for patients, such options are unfeasible with bilateral kidney involvement. Therefore, a chemotherapy regimen, similar to that for multiple myeloma, was determined to be most reasonable. Treatment consisted of 4 cycles of a bortezomib, cyclophosphamide, and dexamethasone regimen. Three months following chemotherapy, kidney function returned to baseline levels.
... Because of the relatively high radiosensitivity, radiotherapy leads to high local control (LC) rate, ranging from 80% to 100%. A substantial rate of cured patients (range 55-87%) is also recorded because of the low rate of progression to multiple myeloma (MM) [2][3][4][5][6][7][8][9][10]. ...
... The only observed exception was the relatively high frequency of the regional lymph node involvement, reaching almost 30%. By comparison, data provided by other authors largely oscillate between 0% and 15% [7,8,[11][12][13][14][15][16]. ...
... The only outfield local recurrence was observed in female patient (6.4 years after radiotherapy), treated effectively with local excision. Similar LC (67-100%) was reported by other authors [2,[4][5][6][7][8][11][12][13][14][15][16][17][18][19]. ...
Extramedullary solitary plasmacytoma (EMP) is a rare type of malignancy. This paper presents a retrospective review of the experience with EMP at the Krakow Oncology Center.Records of 17 patients with head and neck EMP, treated with definitive radiotherapy between 1976 and 2009, were analyzed. The total tumour dose ranged from 45 to 70 Gy (median 56 Gy). In four patients with partial response after radiotherapy, adjuvant melphalan-based chemotherapy was applied. The median follow-up period was 8.6 years.The treatment was well tolerated. The estimated 10-year overall survival, disease-free survival, and multiple myeloma-free survival were 68.4%, 49.3%, and 55%, respectively. The 10-year local control rate was 90.9%. No in-field local recurrence was observed. During the follow-up, progression into multiple myeloma was observed in five patients, with a mean time to conversion of 24 months. The only factor adversely affecting overall survival on univariate analysis was the age >56 years, whereas a complete tumour regression after radiotherapy was associated with a significant improvement in both disease-free survival and multiple myeloma-free survival.Despite the high effectiveness of local radiotherapy, there is still a significant treatment failure risk due to the EMP conversion into generalized disease. An attempt to identify prognostic factors may facilitate selection of patients with a high risk of progression to multiple myeloma. Copyright © 2015 John Wiley & Sons, Ltd.
... EMP accounts for 3-5% of all plasma cell tumors, with an incidence of only 0.04 cases per 100,000 individuals (2). Approximately 80% of EMPs occur in the upper respiratory tract (3), but they can also occur in numerous other sites (3,4), including the gastrointestinal tract (5,6), brain (7), orbits (8), thyroid gland (9), breasts (10), lungs (11), pleura (12), kidneys (13),bladder (14), urethra (15), ovaries (16), testes (17) and skin (18). Usually, EMPs have no specific clinical manifestations. ...
... They typically present as well-localized submucosal masses or swellings in the fifth to seventh decades of the life, with a male to female ratio of 3:1 (3). As the majority of patients can be cured by local radiotherapy, EMP carries an optimistic prognosis, with only 5-20% recurrence (3,4,5,19). EMP in the vulva is extremely rare (20) and to the best of our knowledge, no case involving the labia majora has previously been reported. ...
... A B the mainstay due to the high radiosensitivity of EMP, particularly in the head and neck area where resection is limited by the anatomical complexity. Several series have reported 80-100% local control rates (4,5,19,(23)(24)(25)(26)(27). The optimal radiation dose recommended by the United Kingdom Myeloma Forum is in the range of 40-50 Gy (22,25). ...
Extramedullary plasmacytoma (EMP) in the vulva is extremely rare. The current study presents, for the first time, a case of EMP in the left labia majora in a 36-year-old patient during pregnancy. A painful 7x4x2-cm mass with ulceration, pus and blood scabs, previously misdiagnosed as vulvar ulcer in a local hosipital, was proven to be an EMP by biopsy. Upon magnetic resonance imaging, the tumor was shown to occur in the left labia majora without lymphadenopathy. A complete multiple myeloma (MM) workup excluded coexistence with MM. The goal of treatment was to eradicate the tumor while synchronously preserving the vulva. Therefore, following the termination of the pregnancy, radiotherapy with a total dose of 4,500 cGy markedly reduced the size of the tumor. An extended local excision of the residual tumor, and anaplasty of the vulva preserved the appearance and function of the vulva to the utmost. No post-operative radiation was administered, as the resection margins were not microscopically involved. There was no relapse, metastasis or progression to MM in a 9-month post-operative follow-up period, but close follow-up and long-term surveillance are required.
... Infrequently, EMP can progress to develop PCM, occurring in about 15% of EMP. [1,4] Two-thirds of patients with EMP are men, and the median age at diagnosis is about 55 years. [2,5] Most EMPs are localized in the mucous membranes of the upper respiratory tract; more rarely, they can affect the GI tract, skin, bladder, LNs, and central nervous system. ...
... [16] Regional relapse develops in approximately 25% of patients, and progression to PCM occurs in about 15%. [1,4] In EMP of the small intestine, only 3 cases were reported to progress to PCM. [17][18][19] Because the progression to PCM has a worse prognosis, regular follow-up is necessary using clinical, laboratory, and radiographic images. [2,20] In summary, we describe 2 cases of EMP in the ileum and duodenum that presented with obstruction of the small intestine. ...
Background:
Extramedullary (extraosseous) plasmacytoma (EMP) of the small intestine is a rare plasma cell neoplasm (PCN) that presents with abdominal pain, nausea, vomiting, etc. We describe 2 cases of EMP of the small intestine that presented with ileus.
Methods:
A 78-year-old woman and 68-year-old man visited our clinic with complaints of abdominal discomfort and obstruction. Abdominal computed tomography (CT) revealed a thickened lesion with multiple enlarged mesenteric lymph nodes (LNs) in the ileum and duodenum. The female patient underwent segmental resection in the ileum. The male patient underwent Whipple's operation in the duodenum.
Results:
Histopathological examination and immunohistochemical staining of resected specimens from the 2 patients confirmed a PCN. In the surgical specimens of 2 cases, immunoglobulin heavy-chain rearrangement was confirmed by polymerase chain reaction amplification, but no Epstein-Barr virus (EBV)-infected cells were found by EBV-in situ hybridization. Bone marrow aspirate and trephine biopsies were negative for the type of PCN. Bone marrow cytogenetics and fluorescence in situ hybridization revealed no abnormalities. Serum β2-microglobulin levels were within normal limits. Additionally, none of the patients showed an M-spike in serum or urine protein electrophoresis. Therefore, the patients were diagnosed with a solitary EMP of the small intestine. The female patient refused treatment. At follow-up 3 months postoperatively, her disease progressed and she newly developed multiple LNs and nodular lesions in the right pelvic side wall. She was treated with dexamethasone. The male patient experienced back pain 25 days after Whipple's operation. Spine series magnetic resonance imaging revealed an intermediate signal intensity mass in the posterior epidural space from T8/9 to T10. The mass was removed, and the same histologic features were identified as duodenal masses. He was treated with dexamethasone and radiotherapy.
Conclusions:
EMPs of the small intestine are easy to overlook because they rarely occur in the small intestine. Although surgery is not required for diagnosis, surgical resection can be a good option for EMPs of the small intestine, instead of local radiation therapy. However, close follow-up is required due to the possibility of relapse or progression to plasma cell myeloma.
... The risk factors for multiple myeloma are not conclusive, because the cause of multiple myeloma is not known. Researchers believe that multiple myeloma is most likely the result of many risk factors acting together and may be associated with a decline in the immune system, age, specifi c occupations, exposure to certain chemicals (pesticides, dioxins), HIV infections and exposure to radiation (1,4). ...
... Multiple myeloma presents in the disseminated form, affecting several bones (vertebral bodies, ribs, calvarium bone, pelvis) (1,2,4). Its occurrence in the jaw is common and frequently occurs in the advanced stage of the disease. ...
A neoplastic proliferation of B cell lymphocyte is called plasma cell neoplasms, results from malignant plasma cells transformation in bone marrow. The authors present a clinical study and overview of this pathology in maxillofacial region for six years (Tab. 2, Ref. 14). Text in PDF www.elis.sk.
... Sinonasal tract (47%), nasopharynx (15%), and oral cavity (10%) were the most common primary sites for solitary EMP [3,4,6,7,11,[13][14][15][16][17][18][19][20][21][22][23][24][25][26]. Similarly, sinonasal tract consisted of 69% of primary sites in our series. ...
... A total dose of 40-50 Gy is recommended by most authors. Surgery may have role in selected cases [2,3,8,10,11,15,23,24,27,28,31,33]. The average median total radiation dose used for 499 patients in 22 studies was 44.5 (range 35-52.6) ...
. This study aimed to report the characteristics and treatment outcome of 13 patients with solitary extramedullary plasmacytomas of the head and neck and analytical literature review.
Materials and Methods
. Nine patients (69%) were treated with gross surgical resection followed by radiotherapy, three (23%) were primarily treated with radiotherapy alone, and one (8%) was treated with surgery alone.
Results
. There were 3 women and 10 men with the median age of 52 years. Nasal cavity (46%) and paranasal sinuses (23%) were the most common primary sites. After a median follow-up of 28 months, 10 patients are alive and free of disease, one is alive with multiple myeloma, and two died of multiple myeloma. In the literature review the median age was 58 years and male/female ratio was 2.7. Sinonasal tract was the most common primary site. Ten-year local control and progression to multiple myeloma rates were 88% and 23%, respectively. The 5- and the 10-year overall survival rates were 71 and 69%, respectively.
Conclusion
. Radiation therapy with or without surgery is an effective treatment for patients with head and neck extramedullary plasmacytoma. However, long-term follow-up for detection of local recurrence and progression to multiple myeloma is essential.
... Fractionated radiotherapy dose of 40-50 Gy over a duration of 4 weeks is given at the rate of 1.8 to 2.0 Gy per fraction [12]. Chao et al. found that surgical resection, either alone or in combination with adjuvant irradiation, proved a better pattern of response to EMPs originating in the head and neck [14]. The role of angiogenesis inhibitors, thalidomide, protease inhibitors, or inhibitors of vascular endothelium growth factor in plasma cell neoplasms is now the subject of further research. ...
Plasmacytomas are localized monoclonal plasma cell lesions with no evidence of systemic involvement which are divided into solitary bone plasmacytoma (SBP) and extra-medullary plasmacytoma (EMP). The diagnosis of plasmacytomas (PCM) in the oral regions is challenging given the atypical clinical manifestations and low frequency. Here, we report an extremely rare case of plasmacytoma in an elderly male which initially appeared to be arising from the left buccal mucosa on clinical examination but after radiological imaging and intra-operative findings, the epicentre was found to be in the left infratemporal fossa (ITF). The patient underwent en-bloc compartment resection with high clearance of the ITF which proved to be an effective management strategy. It is crucial for the head and neck surgeon to be aware of the solitary bone plasmacytoma in the oral and maxillofacial region in order to identify it early and provide these patients with the best care possible before complications arise.
... Chao MW., et al. [10] reported a patient with multiple plasmacytoma without progression into multiple myeloma in 14 years period evaluation. Early treatment can reduce the local complication of disease [11,12]. ...
... Approximately 70% of patients with solitary extramedullary plasmacytoma remain disease-free at 10 years. [3,14,16,17]. ...
Extramedullary (extraosseous) plasmacytoma represents a rare disease that accounts for less 5% of plasma cell neoplasms. It commonly occurs in the upper respiratory tract, with 80% of cases involving the paranasal sinuses, nasopharynx, or nasal cavity. Plasmacytomas of the eyelid are very rare with only ten cases having been reported in the literature so far. We present the clinical and histopathologic findings from a case of plasmacytoma of the eyelid and discuss its histopathologic differential diagnoses. To our knowledge, this is the first case of plasmacytoma of the eyelid in an HIV-positive patient in the English literature
... Chao ve arkadaşları 16 soliter ekstramedüller plasmositom olgusunun tamamını radyoterapi ile tedavi etmişlerdir. Ortalama 5 yıllık izlemde yerel yineleme iki olguda izlenmiştir (6). ...
ZET 50 yaşında erkek hastaya sağ gözde iki hafta içinde oluşan kitle nedeniyle biyopsi uygulandı. Işık mikroskobi ve imundokukimyasal boyamada plasmositom tanısı kondu. Sistemik taramada multipl myelom tanısı doğrulandı. Olguya multipl myeloma bağlı gelişen orbital plasmositom tanısı kondu. Radyoterapi ve kemoterapi uygulandı. Orbital kitle şeklinde ortaya çıkan nadir bir sekonder orbital plazmositom olgusunu sunmak istedik. Anahtar Sözcükler: Multipl miyelom, Orbital kitle, Plazmositom SUMMARY A 50 years old man presented with a growth mass in the right eye of two weeks duration. Biopsy of the mass followed by light microscopy was suggestive of plasmacytoma and the diagnosis was confirmed by immunohistochemical stains. Systemic work up was positive. The diagnosis of secondary orbital plasmacytoma was made. The patient received local radiotherapy and chemotherapy. As it is seldomly seen, a case of secondary orbital plasmacytoma presenting as an orbital mass is reported.
... We have compensated for this limitation by following up with our patients for a median of 7 years, which is relatively longer than most studies in the literature. 24,27,[63][64][65][66] CONCLUSIONS In our study, we observed that a sudden onset of neurological deterioration had led predominantly to surgery. Our patients benefited from surgical treatment in terms of good short-and long-term pain reduction. ...
Introduction:
Multiple myeloma (MM) is the most frequent primary malignancy of the spine. We aimed to investigate the clinical presentation, surgical indications and outcomes, complications, survival, and its influencing factors in surgically treated MM patients with symptomatic involvement of the spine (SIS).
Methods:
Retrospective analysis of prospectively collected data. Out of 350 MM patients treated at our institution over a period of 12 years (2006-2018), we identified 24 patients who were surgically treated for SIS. We collected data on demographics, clinical presentation, comorbidities, surgical indications, and outcomes and investigated the factors predisposing to postoperative complications and survival.
Results:
The median follow-up duration was 85 months; median overall survival (OS) was 50 months. Clinical presentation at admission included pain (88%), sensory and/or motor deficit (67%), and bowel/bladder dysfunction (25%). Symptomatic pathological fractures were seen in 33%. Predominant surgical indications were rapid neurological deterioration with or without spinal cord compression (SCC), followed by mechanical instability. The majority of our patients benefited from surgery in terms of pain reduction in the short term as well as in the long term. There were 21% patients with surgical-related complications (<3 months). Surgical site infections occurred in 17%, without any obvious factors predisposing to infective complications. Neurological deterioration during hospital stay, especially in the presence of motor deficit and/or bowel/bladder dysfunction, significantly reduced OS.
Conclusions:
Sudden-onset neurological deterioration was the predominant factor leading to surgery. We achieved good short- and long-term pain reduction. Surgery is a valuable option for MM patients with SIS who present with rapid neurological deterioration with or without SCC and/or mechanical instability.
... Previous studies showed that approximately 50% of patients with SBP and 15% with EMP will progress to MM [11,12]. We confirmed these data in terms of prevalence and rate, which are higher in SBP than in EMP. ...
We retrospectively analyzed 51 patients with solitary plasmacytoma diagnosed from October 2002 to September 2018 from a cohort of 3575 patients with plasma cell dyscrasias registered in the Kansai Myeloma Forum. Twenty-seven patients had solitary bone plasmacytoma (SBP) and 24 had extramedullary plasmacytoma (EMP), with prevalence of 0.8% and 0.7%, respectively. The most frequent M protein was IgG (40%) in SBP, whereas non-secretory proteins were most frequent (50%) in EMP. Five-year overall survival was 78.2% in SBP and 80.8% in EMP (P = 0.894). Among patients with SBP, 44% progressed to MM with a median time of 10.5 months (2.4–93.3 months), whereas 8% of EMP patients progressed to MM with a median time of 18.6 months (13.0–24.2 months). The most frequent treatment was radiotherapy (41%) or observation (41%) in SBP, and chemotherapy (54%) in EMP. No statistically significant difference was observed upon univariate analysis of prognostic factors including age, sex, performance status, and IgG M protein. Our results suggest that there are biological differences between SBP and EMP in real-world settings.
... While some authors suggest surgery as the first choice (11,26,28) , Radiation therapy (40 -50 Gy delivered to tumor site) remains as the primary treatment for SBP with local disease control in 80% of the cases (6). Surgical treatment is not the preferred treatment in most instances and remains for patients with well-defined SBPs and minimal cosmetic or functional deficit. ...
Introduction: Plasma cell tumors are monoclonal neoplastic proliferation of plasma cells which are divided into three groups: solitary bone plasmacytoma (SBP) and extramedullary plasmacytoma (EMP) which are localized forms, and multiple myeloma (MM) which is the disseminated form. Skull, long bones, and vertebrae are common sites for SBP and its presence in maxillofacial area is rare. There is a possibility that SBP converts to MM and some authors believe that it can be the early stage of MM. Case Presentation: Here, we present a 59-year old male patient with a mass in maxillary bone enlarging after tooth extraction. Cone beam computed tomography (CBCT) confirmed the existence of a poorly defined lesion in the left maxilla. The microscopic evaluation and immunohistochemical studies confirmed the diffuse and monoclonal population of cells, indicating the diagnosis of plasma cell tumor. There was also no evidence of bone marrow involvement in the aspiration. Conclusions: Early and precise diagnosis of SBP is a crucial element to rule out the possibility of MM. It must be kept in mind that there is possibility of recurrence or progression to MM after treatment which can completely change the course of treatment, and this emphasizes the importance of regular follow up.
... Radiotherapy is the treatment of choice for extramedullary plasmacytoma, presenting a local control rate of 90-100% 23,24 . There is no consensus regarding the recommended optimal dose; doses with an optimal control rate can range from 35 to 80 Gy for a period between 3 and 6 weeks 4,12,25-27 . ...
Plasmacytoma is a rare, non-epithelial, malignant tumor, affecting soft or bony tissues, which can have a unique location (solitary or extramedullary solitary plasmacytoma), or multiple (multiple myeloma). In the ENT sphere, it is most commonly encountered in its extramedullary form, which develops in the region of the head and neck, at the level of the submucosa. Most commonly, it affects the elderly, who have cardinal symptoms such as unilateral nasal obstruction, nasal or facial swelling, tissue necrosis or mucosal ulcerations, perforation of the nasal septum with dysmorphia of the nasal pyramid, recurrent unilateral epistaxis. Radiotherapy is the treatment of choice for extramedullary plasmacytoma. The prognosis is a reserved one, the risk of recurrence or conversion to multiple myeloma also existing 15 years after treatment.
... 14 In general, EMPs are considered radiosensitive, with a local control rate of 90 to 100%. 16 A radiation dose of 40 to 50 Gy delivered to the primary site of the EMP in the nasopharynx is usually recommended. 17 Irradiation to the neck is required only in cases with clinically positive cervical node metastasis. ...
Plasmacytoma is an extremely rare and discrete solitary mass of neoplastic monoclonal plasma cells. Extramedullary plasmacytoma tends to occur during the fifth and seventh decades of life and is rarely diagnosed in younger patients. In this study, a 55-year-old patient presented to us with complaint of changes in voice, and difficulty in swallowing and breathing. On examination, there was a retropharyngeal mass which is extending to lateral pharyngeal wall and toward nasopharynx. Contrast computed tomography scan shows soft tissue density involving retropharyngeal space and involving nasopharynx and left parapharyngeal space. For this, the patient underwent excision of this mass under General Anaesthesia (GA) biopsy report was suggestive of plasmacytoma. After excluding systemic involvement by means of laboratorial and radiological investigations, the patient was referred to radiotherapy department for complete management.
How to cite this article
Gupta R, Sharma D, Minhas RS. Extramedullary Plasmacytoma of Soft Tissue Neck. Int J Phonosurg Laryngol 2016;6(1):42-46.
... But it is clinically important to distinguish anaplastic EMP and PBL because the optimal therapies and clinical courses are very different. Local radiotherapy is the preferred therapeutic modality for primary EMP owing to its radio-sensitivity (9). A surgical resection of the mass is also another good option. ...
We herein present the case of an immunocompetent 63-year-old man who had previously undergone resection of Crohn's disease (CD)-related small intestinal obstruction more than 30 years ago. He had not been receiving any medication for many years, but had recently started to suffer from ileus. A stenosed site of ileo-cecal anastomosis was identified and therefore was surgically resected, which was diagnosed as CD with small intestinal extramedullary plasmacytoma (EMP). The subsequent progression of CD was successfully controlled by anti-TNFα agents without any recurrence of EMP for over 3 years, implying the clinical benefit and safety of the biological therapy. This was the first known case of a patient who received anti-TNFα agents after a resection of small intestinal EMP accompanied with CD.
... For most EMPs occur in upper aerodigestive tract, which is difficult to have an operation, local radiotherapy is the preferred therapeutic modality. [24] However, the instant surgical resection of the mass is another good option for gastrointestinal EMP, having the lowest recurrence rate. The previous investigation provides evidence that surgery alone gives the best results in cases of EMP when it is resectable. ...
Rationale:
Extramedullary plasmacytomas (EMP) are tumors composed by a monoclonal population of plasma cells that arise in extraosseus tissues, occupying <5% of all plasma cell neoplasms. Gastrointestinal solitary extramedullary plasmacytoma is rare, just comprises about 5% of all EMPs. The most common site is small intestine. The gastric incidence is much rare and especially the plasmablastic plasmacytoma in the stomach.
Patient concerns:
A 65-year-old man had an epigastric discomfort and mass for about 2 months. Gastroscopy revealed a malignant tumor in the gastric body. Abdomen computed tomography (CT) showed that the gastric cavity was filled, and the irregular soft tissue shadow was seen in the greater curvature, and the enhancement was obvious. To get more tissue, we conducted stomach puncture biopsy. Pathology showed small-round cell malignant tumors. And immunohistochemical examinations revealed that the tumor tend to be a plasma cell tumor.
Diagnosis:
Gastric plasma cell tumor.
Interventions:
Distal gastrectomy was performed to treat the tumor.
Outcomes:
In addition to ascites caused by hypoproteinemia, there were no postoperative complications. Postoperative pathologic report showed plasmablastic plasmacytoma. Histopathologic examination of the specimen revealed plasmablastic plasmacytoma originating in the stomach with transmural extension, but without lymph node metastasis. The patient is regularly followed up at a postoperative clinic and is doing well, and at present there is no plan for adjuvant treatment.
Lessons:
Surgical resection is good option for gastrointestinal EMP.
... Usually, EMPs are very sensitive to radiotherapy, and complete remission can be achieved by radiotherapy alone in 80% to 100% of cases. 6,11,15 Surgery is considered if the tumor is di use or is causing symptoms secondary to pressure on surrounding structures. A combined approach is recommended in cases with incomplete surgical margin or lymph node involvement. ...
Extramedullary plasmacytoma of the thyroid, refractory to radiation therapy and treated with bortezomib P lasma cell neoplasms involving tissues other than the bone marrow are known as extra-medullary plasmacytoma (EMP). 1 EMPs mostly involve the head and neck region. 2 Solitary EMP involving only the thyroid gland is very rare. 3,4 Because of the limited knowledge about this condition and its rarity, its management can be challenging and is often extrapolated from plasma cell myeloma. 5,6 In general, surgery or radiation are considered as front-line therapy. 3,5 EMPs usually respond well to radiotherapy with almost complete remission. No deenite guidelines outlining the treatment of radio-resistant EMP of the thyroid have yet been published. Data supporting the use of chemo-therapy is particularly limited. 4,7,8 Here, we describe the case of a 53-year-old woman with a long history of thyroiditis who presented with rapidly worsening symptomatic thyroid enlargement. She was diagnosed with EMP of the thyroid gland that was not amenable to surgery and was refractory to radiotherapy but responded to adjuvant chemotherapy with bortezomib. is report highlights 2 unique aspects of this condition: it focuses on a rare case of EMP and, as far as we know, it reports for the rst time on EMP that was resistant to radiotherapy. It also highlights the need for guidelines for the treatment of EMPs. Case presentation and summary A 53-year-old woman presented to the emergency department with complaints of diiculty swallowing , hoarseness, and neck pain during the previous 1 month. She had a known history of Hashimoto's thyroiditis, and an ultrasound scan of her neck 6 years previously had demonstrated diiuse thyro-megaly without discrete nodules. On presentation, the patient's vitals were stable, and a neck examination revealed a rm and enlarged thyroid without any cervical adenopathy. Laboratory investigations revealed a normal complete blood count and comprehensive metabolic panel. She had an elevated thyroid-stimulating hormone level of 13.40 mIU/L (reference range, 0.47-4.68 mIU/L) and normal thyroxine level of 4.5 pmol/L (reference range, 4.5-12.0 pmol/L). A computerized tomog-raphy (CT) scan of the neck revealed an enlarged thyroid gland (right lobe length, 10.3 cm; isthmus, 2 cm; left lobe, 8 cm) with a focal area of increased echogenicity in the midpole of the left lobe measuring 9.5 mm × 5.5 mm. e patient was discharged to home with pain medications, and urgent follow-up with an otolaryngologist was arranged. A ex-ible laryngoscopy was done in the otolaryngology clinic, which revealed retropharyngeal bulging that correlated with the thyromegaly evident on the CT scan. Because of the patient's signiicant symptoms, we decided to proceed with surgery with a clinical diagnosis of likely thyroiditis. A left subtotal thy-roidectomy with extension to the superior medi-astinum was performed, but a right thyroidectomy could not be done safely. On gross examination, a well-capsulated left lobe with a tan-white, lobu-lated, soft cut surface was seen. Microscopic examination revealed replacement of thyroid parenchyma with sheets of mature-appearing plasma cells with eccentric round nuclei, abundant eosinophilic cyto-plasm without atypia, and few scattered thyroid fol-licles with lymphoepithelial lesions (Figure 1A). Immunohistochemistry connrmed plasma cells with
... Usually, EMPs are very sensitive to radiotherapy, and complete remission can be achieved by radiotherapy alone in 80% to 100% of cases. 6,11,15 Surgery is considered if the tumor is di use or is causing symptoms secondary to pressure on surrounding structures. A combined approach is recommended in cases with incomplete surgical margin or lymph node involvement. ...
... SBP most frequently occurs in the axial skeleton, such as a vertebra, while SEP is observed in the head and neck, and these two entities have a clinical course and prognosis that are quite different from each other. 2,3,[5][6][7][11][12][13][14][15][16][17] All these data reported are prevalently based on historical studies; there are few series reported in the most recent years. However, last year, de Waal et al. reported the experience between 1988 and 2011 in the northern area of the Netherlands about 76 SP patients, confirming the historical data. ...
Solitary plasmacytoma is a rare disease characterized by a localized proliferation of neoplastic monoclonal plasma cells, without evidence of systemic disease. It can be subdivided into solitary bone plasmacytoma if the lesion originates in bone, or solitary extramedullary plasmacytoma if the lesion involves a soft tissue. The incidence of solitary bone plasmacytoma is higher than solitary extramedullary plasmacytoma. Also, the prognosis is different: even if both forms respond well to treatment, overall survival and progression-free survival of solitary bone plasmacytoma are poorer than solitary extramedullary plasmacytoma due to its higher rate of evolution in multiple myeloma. However, the recent advances in the diagnosis of multiple myeloma can better refine also the diagnosis of plasmacytoma. Flow cytometry studies and molecular analysis may reveal clonal plasma cells in the bone marrow; magnetic resonance imaging or 18 Fluorodeoxyglucose positron emission tomography could better define osteolytic bone lesions. A more explicit exclusion of possible occult systemic involvement can avoid cases of misdiagnosed multiple myeloma patients, which were previously considered solitary plasmacytoma and less treated, with an unavoidable poor prognosis.
Due to the rarity of the disease, there is no uniform consensus about prognostic factors and treatment. Radiotherapy is the treatment of choice; however, some authors debate about the radiotherapy dose and the relationship with the response rate. Moreover, the role of surgery and chemotherapy is still under debate. Nevertheless, we must consider that the majority of studies include a small number of patients and analyze the efficacy of conventional chemotherapy; few cases are reported concerning the efficacy of novel agents.
... As it considered radiosensitive, with a local control rate of 90-100% with a rate of conversion into MM is 31%. Moderate dose RT of at least 40 Gy using limited radiation fields is recommended [14,15,17,19,20]. Because of the high rate of recurrence and progression to multiple myeloma, followup radiological and electrophoresis assessment is required following treatment [22]. ...
Key Clinical Message
Plasma cell neoplasms are characterized by a neoplastic plasma cell lineage which produces a monoclonal immunoglobulin. These neoplasms can present as a single lesion (solitary plasmacytoma) or as multiple lesions (multiple myeloma). Solitary plasmacytomas most frequently occur in bone (plasmacytomas of bone), but can also be found outside bone in soft tissues (extramedullary plasmacytomas).
... Primary SBP and EP have been classified as distinct clinical entities within the plasma cell dyscrasia family by both the World Health Organization and International Myeloma Working Group [1,2] and have a relatively good prognosis with local radiotherapy alone with 50-72% of SBP and 0-36% EP 10 years progression to myeloma. [3][4][5][6][7][8][9][10][11][12][13] Regardless of the association with underlying MM, plasmacytoma of the testis is very uncommon. [14,15] Seventy-one cases of testicular plasmacytoma have been published up to 2008. ...
Testicular plasmacytoma, whether occurring as a primary lesion or as a reflection of underlying multiple myeloma (MM), is a rare disease. We report the case of a 38-year-old male with multiply relapsed MM, who was found to have a testicular plasmacytoma. He presented with a gradually enlarging scrotal mass. Following orchidectomy, pathologic examination of the specimen demonstrated a plasmacytoma. In the context of active MM, the specimen was also sent for cytogenetic analysis but this was unhelpful in guiding a chemotherapy regime, which still continues at time of reporting. Although a rare lesion, there remains no definitive treatment protocol for the management of testicular plasmacytoma representing an extramedullary manifestation of MM.
... Treatment options include surgery, chemotherapy and radiotherapy, either alone or combination (5). Local radiotherapy is the preferred therapeutic modality for EMP owing to its documented radiosensitivity (6). However, previous studies indicated that a combination treatment may provide the best results (7). ...
Multiple myeloma (MM) is a rare hematological malignancy, characterized by uncontrolled proliferation of plasma cells in the bone marrow. MM is usually confined to the bone marrow, however, it may occasionally infiltrate other tissues, which is known as extramedullary plasmacytoma (EMP). The majority of EMPs involve the head and neck region, although different anatomical sites, including the gastrointestinal tract, central nervous system, thyroid gland and breast may also be affected. The simultaneous presentation of EMP in the kidney and head is rare, presenting diagnostic challenges due to its unusual location and non-specific or absent symptoms. To the best of our knowledge, no case of extramedullary plasmacytoma presenting with simultaneous renal and intracranial infiltration has been reported in the literature thus far. However, the present study reports a case of primary renal and intracranial extramedullary plasmacytoma in a 76-year-old male patient. The patient presented with a swelling over the right side of the forehead, which had slowly increased in size prior to hospital admission. The swelling was associated with dizziness and weakness, without bone pain. Contrast magnetic resonance imaging suggested an osteolytic skull lesion with intracranial extension. Abdominal enhanced computed tomography scanning revealed a large tumor mass extending around and into the kidneys. Immunohistochemical examination of the renal tumor biopsy, and blood and serum samples, as well as immunoelectrophoresis of serum proteins, resulted in a diagnosis of EMP being proposed. Therefore, the patient was administered with two cycles of cyclophosphamide and thalidomide in combination with dexamethasone. Follow‑up imaging performed 4 months later revealed almost complete disappearance of the intracranial tumor mass and renal infiltration. The current study also presented a review of the literature. This study revealed that EMPs may co‑exist with MM or present as the main symptom of MM. The diagnosis of an EMP is complex and requires radiological, hematological, biochemical and histological investigation. At present, no guidelines for EMP treatment have been established and thus, treatment options include surgery, chemotherapy and radiotherapy, either alone or in combination. We hypothesize that combined treatment may provide the best patient outcome.
... W leczeniu odosobnionej postaci szpiczaka naleŜy rozwaŜyć zabieg chirurgiczny, radioterapię oraz w wybranych przypadkach chemioterapię. Wysoka radiowraŜliwość guzów pozakostnych pozwala na wyleczenie i uniknięcie okaleczenia chorego, zwłaszcza, kiedy zmiana umiejscowiona jest w obrębie głowy i szyi [3,10]. Zastosowanie umiarkowanych dawek radioterapii umoŜliwia uzyskanie miejscowej remisji w 80-100% przypadków [7,8]. ...
SUMMARY
Extramedullary plasmacytoma (EMP) is a plasma cell tumor arising outside of the bone marrow, particularly (80–90%) the head and neck region (nasopharynx, nose cavity, sinuses, and tonsils), and can be associated with multiple myeloma (MM). EMP is an uncommon neoplasm and rarely occurs in the breast. We report a 74-year-old female with a EMP in the left breast associated with MM and discuss its clinical features, diagnosis and treatment.
... Since EMP, as well as solitary bone plasmacytoma, is a highly radiosensitive neoplasm, the treatment of choice is radiotherapy given at the dose of 35-45 Gy. Chemotherapy and surgical procedures are rarely necessary because no clear evidence exists that they can reduce the probability of progression to multiple myeloma or increase disease-free survival [Weber, 2005;Chao, 2005]. ...
We describe the case of a patient who received the diagnosis of squamous cell carcinoma of the radio-treated skin of the sacrococcygeal region where an extramedullary plasmacytoma had been identified one year before. We think that the plasmacytoma was born by a malignant transformation of a host-inflammatory reaction to a not-detected epithelial tumor. It can also hypothesized that plasma-cell dyscrasia or post-radiation infiammatory reaction had promoved the squamous cell carcinoma occurrence.
Extramedullary plasmacytomas (EMPs) are solitary plasma cell neoplasms that involve sites other than bone or bone marrow. It constitutes only 0.4% of tumors of head-and-neck region. Surgery and radiotherapy are standard treatment, depending on the site. Radiotherapy promises 90–100% local control rate and prolonged survival. Here, we are reporting a case of plasmacytoma nasopharynx treated with volumetric-modulated arc technique (VMAT) radiotherapy, with contouring details, dose to target, and nearby structures. Patient had complete response and is relapse free for the past 3 years with minimal toxicity. In our case, the lesion is in nasopharynx and is about 1cm, as such it required precise radiotherapy technique like VMAT, to give targeted dose delivery and sparing the nearby critical organs.
The involvement of the larynx in plasma cell myeloma (PCM) may manifest as solitary extramedullary plasmacytoma of the larynx (sEMP-L) or as infiltration of the larynx during newly diagnosed or relapsed systemic disease with bone marrow involvement (plasma cell myeloma with laryngeal involvement, PCM-L). To increase knowledge about these rare conditions, we performed a retrospective analysis along with a comprehensive literature review of cases of sEMP-L or PCM-L. Six patients (two sEMP-L and four PCM-L) were identified in our tertiary laryngological centre from 2009 to 2021, constituting 0.88% of all malignant laryngeal tumours. The literature search yielded 187 cases, including 152 sEMP-L and 35 sPCM-L. A comparison of baseline characteristics between sEMP-L and PCM-L performed in the combined cohort of cases from literature review and retrospective analysis revealed that patients with sEMP-L were younger (56 vs. 64 years, p ≤ 0.001) and presented less commonly with thyroid or cricoid cartilage involvement (2.2% vs. 30.8%, p ≤ 0.001). The prognosis of sEMP-L was better than PCM-L (overall survival 86% vs. 55% at 5 years, p = 0.002). Analysis of potential factors that could influence progression-free survival (PFS) in the group of sEMP-L revealed that male sex and cartilage involvement negatively affected PFS in univariate analyses, while only cartilage involvement retained statistical significance in multivariate analysis (HR = 19.94, p = 0.024). In conclusion, PCM with laryngeal involvement is sporadic. Secondary involvement of the larynx during PCM might be more common than sEMP-L and is associated with worse survival. The involvement of cartilage adversely influences the outcome of sEMP-L.
Objective
To analyze the factors related to postoperative recurrence in patients with localized laryngeal amyloidosis (LocLA) and to construct a nomogram prediction model (NPM).
Methods
We collected the data for LocLA patients diagnosed from March 2000 to May 2019 and clinical characteristics data were extracted. Factors related to recurrence were analyzed using multivariate logistic regression. The NPM was constructed for predicting the recurrence risk of LocLA. The receiver operating characteristic (ROC) curve evaluated the distinguishing ability using the area under curve (AUC). The calibration curve was created to evaluate the consistency of the NPM.
Results
A total of 226 confirmed LocLA cases were included. One hundred seventy-five cases (77.4%) had localized single nodule, and 51 cases had more than one lesions. Sixty-three (27.9%) cases had no multinucleated giant cell (MGC) around amyloid, and 163 (72.1%) cases had MGC around amyloid. Multivariate logistic regression analysis showed that more than one lesions (odds ratio [OR] = 3.206 and 95% confidence interval [CI]: 1.492-6.888; P value: .003), subglottic involvement (OR = 2.926 and 95% CI: 1.300-6.585; P = .010), and no multinucleated giant cell (MGC) around amyloid (OR = 2.503 and 95% CI: 1.173-5.342; P = .018) had a statistically significant effect on postoperative LocLA recurrence ( P < .05). The AUC of the ROC curve was 0.753 (95% CI: 0.667-0.832). The bias-corrected curve approached the ideal curve, with an average absolute error of 0.037.
Conclusions
More than one lesions, subglottic involvement, and no MGC around amyloid are risk factors for postoperative recurrence of LocLA. The NPM constructed has good applicability.
Plasma cell dyscrasias are a group of diseases with varying severity ranging from those causing systemic malignancy such as Multiple Myeloma, to clinically benign pathologies, such as solitary plasmacytoma of bone. Solitary plasmacytoma is a rare localized form of neoplastic proliferation of monoclonal plasma cells that produce a single osseous lesion. It accounts for 3% of all plasma cell malignancies, with the most common location being the axial skeleton. A literature review reveals very few reported cases of solitary plasmacytoma in the foot and ankle. We searched through multiple databases including the Journal of Foot and Ankle Surgery as well as PubMed Central using key words such as “plasmacytoma” and “plasma cell dyscrasia.” Involvement of the lower extremity is extremely rare. We report a case of plasmacytoma of the Talus with subsequent pathologic fracture, highlighting the unusual clinical features of presentation while exploring diagnostic modalities for solitary plasmacytoma in the foot. To our knowledge, only one other plasmacytoma findings in the foot and ankle have previously presented with a pathologic fracture. This case report shows radiographic and advanced imaging findings.
Background: Tongue extramedullary plasmacytoma is a very rare pathology. Despite rare cases, extramedullary plasmacytoma should be considered as a differential diagnosis in case of a mass in the tongue. A total of 19 cases were reported with EMP in English literature along with the case we will address. It is characterized by a monoclonal neoplastic proliferation of plasma cells in the absence of multiple myeloma (MM). Histopathology and immunohistochemistry are very important for the diagnosis and differential diagnosis.
Background: Tongue extramedullary plasmacytoma is a very rare pathology. Despite rare cases, extramedullary plasmacytoma should be considered as a differential diagnosis in case of a mass in the tongue. A total of 19 cases were reported with EMP in English literature along with the case we will address. It is characterized by a monoclonal neoplastic proliferation of plasma cells in the absence of multiple myeloma (MM). Histopathology and immunohistochemistry are very important for the diagnosis and differential diagnosis.
This article discusses electrophoretic patterns and methodologies, especially descriptions of the methods with some historical content, weaknesses of the techniques and exact recommended collection and procedural processes. Many electrophoretic patterns are presented as an introduction to those who are gaining familiarity with the techniques and as advanced information for practitioners. The basis of humeral immunology is discussed to improve the understanding of where, why and how monoclonal gammopathies develop. Methodological topics discussed include serum and urine electrophoresis and immunoelectrophoresis, nephelometric and turbidometric measurement, serum free light chain assays, cryoglobulins isolation and measurement, interference from circulating immune complexes and other interferences. Clinical topics discussed include multiple myeloma, including both active and asymptomatic (smoldering), monoclonal gammopathy of undetermined significance, amyloidosis, Waldenstrom macroglobulinemia, cryoglobulinemia, circulating immune complexes, and POEMS syndrome as well as other neurological conditions associated with monoclonal proteins. Criteria for diagnosis defined by the international Myeloma Working Group are discussed. There is also an over view of associated workup for diagnosing, monitoring progress and therapy and defining status of persons with or suspected of having monoclonal gammopathies, and some information on treatments to put the concepts in a more complete perspective. The article also focused on why these methods are implicit in diagnosing, monitoring, treating and defining status of these conditions.
Extramedullary plasmacytoma (EMP) represents a distinct yet rare entity among the plasma cell neoplasms. Given its rarity, no therapeutic consensus has been met. We report the case of a 57-year-old man with a one-year history of nasal congestion and occasional dyspnoea. Imaging showed a hypermetabolic mass in the right nasopharynx extending backward towards the adjacent oropharynx, infiltrating the epiglottis. As incisional biopsy showed histologic and immunophenotypic features consistent with plasma cell neoplasm, whereas the possibility of a marginal zone lymphoma with plasmacytic differentiation was included in the differential diagnosis. A final diagnosis of EMP was reached by using flow cytometry (FC) of a cell suspension from the neoplastic tissue. The patient received local radiotherapy (RT) which resulted to complete remission. In conclusion, flow cytometry might serve as an auxiliary method in cases where immunohistochemistry cannot differentiate between a plasma cell dyscrasia and a B-non-Hodgkin lymphoma. In cases of an established diagnosis of solitary nasopharyngeal EMP RT represents an excellent treatment modality offering prolonged disease-free survival.
Tumors are unfortunately relatively common in the large intestine, and colorectal cancer is the second or third leading cause of cancer-related death in the United States alone, depending on the year. Adenocarcinoma is the most common type, comprising approximately 95%, with various other subtypes accounting for the rest. The latter group of rare tumors of the colon and rectum can be broadly separated into one of four categories: epithelial, lymphoid, mesenchymal, or other. In this chapter we describe the presentation, diagnosis, and treatment of these different tumor types.
Objectives:
Laryngeal amyloidosis (LA) is a rare disease characterized by extracellular protein deposition within the larynx. Treatment is difficult due to the frequently submucosal and multifocal nature of disease. The mainstay of treatment is surgical resection; however, recurrence rates are high. Recently, use of radiotherapy (RT), either alone or postoperatively, for LA has been adapted from the management of extramedullary plasmacytoma and has been shown to provide local disease control. Here, we describe the experience with adjuvant RT for LA at our center.
Study design:
Retrospective case series.
Methods:
Retrospective study of patients with amyloidosis of the larynx, with or without other disease sites, seen at a tertiary academic center between 2011 and 2019. Outcomes included disease characteristics, recurrence rates, treatment modalities, and pre- and posttreatment voice handicap index (VHI)-10.
Results:
Ten patients met eligibility criteria. Mean follow-up time for all patients was 62.0 ± 41.0 months; mean follow-up time after last treatment was 51 ± 55 months. All but one patient underwent surgical resection of disease. Seven patients underwent subsequent RT. Of these seven, six underwent RT at our institution; five received a dose of 45 Gray (Gy); and one received a dose of 20 Gy. All seven completed RT without toxicity-related interruption. Patients undergoing RT underwent 2.1 ± 1.3 surgical procedures prior to RT; no patients required surgery after RT. Mean pretreatment VHI-10 was 22.9 ± 8.1; mean posttreatment VHI-10 was 12.9 ± 13.3.
Conclusion:
RT after surgery for LA can provide good local control without unacceptable toxicity and may decrease the need for further surgery.
Level of evidence:
IV. Laryngoscope, 2020.
Objective:
Definitive radiotherapy (RT) with or without surgery is the standard of care for solitary plasmacytoma. Here, we report clinical outcomes for this rare malignant neoplasm.
Patients and methods:
We retrospectively reviewed the medical records of adults with solitary plasmacytoma treated with definitive RT between 1963 and 2015 at a single institution, and assessed disease control, survival, and toxicity per Common Terminology Criteria for Adverse Events (CTCAE), version 4.
Results:
A total of 42 patients with solitary plasmacytoma of the bone (SPB, n=27) or extramedullary plasmacytoma (EMP, n=15) were treated with definitive RT with (n=11) or without (n=31) surgical resection. The median age at diagnosis was 59 years (range: 28 to 76 y).Twenty-two patients had tumors ≥5 cm and 20 had tumors <5 cm. Immunoglobulins were elevated in 23 patients and M-protein in 14. The median RT dose was 45 Gy (range: 15 to 54 Gy) over a median 25 fractions (range: 1 to 38 fractions) with 3 patients receiving twice-daily fractionation and 6 received elective nodal irradiation. No patients received adjuvant chemotherapy. The median follow-up was 10.3 years. The 10-year local control rate after RT was 88%. Five patients who developed a local recurrence had SPB ≥5 cm. The 10-year multiple myeloma-free survival rates were: overall, 47%; SPB, 24%; and EMP, 87% (P=0.0012). The 10-year cause-specific survival rate was 75%: 64% for SPB versus 93% for EMP (P=0.0116). The 10-year overall survival rate was 60%. Three patients experienced late grade 2+ toxicity.
Conclusions:
Definitive RT with moderate doses results in excellent local control. We observed a higher rate of progression to multiple myeloma and lower survival in patients with SPB compared with EMP.
Extramedullary plasmacytoma (EMP) of the larynx is an extremely rare plasma cell neoplasm outside of the bone marrow, which has not been previously well characterized. A case of laryngeal EMP who developed acute myeloid leukemia (AML) following treatment is described in the present study, as well as an extensive review of the relevant literature. An electronic literature search was performed in PubMed and all pertinent case reports and series in the English language from 1948‑October 2017 were identified. A total of 99 cases including the present case were available for review. The mean age of the included patients was 53 years. Supraglottis was the most frequently involved site. The most common treatment modality was radiotherapy alone (n=41; 43%), followed by a combination of surgery and radiotherapy, then surgery alone. However, for cases published in recent years, the most common treatment modality was surgically based treatment. Overall the treatment outcome was favorable, as a total of 84% of patients were alive after a mean follow-up of 60 months. However, EMP outcomes for patients with cervical lymphadenopathy or multiple sites involvement were unfavorable with >40% of patients relapsing or developing metastasis during the limited follow-up period. A total of 6 subjects developed multiple myeloma and 1 patient converted to AML. The present study provides important insights on the treatment of EMP, which is a rare disease. To the best of our knowledge, this is the first case report of a patient with laryngeal EMP who developed AML following treatment. It is recommended that secondary myeloid neoplasm should be considered besides multiple myeloma during the follow-up period.
It has been estimated that there are about 748,3000 cases of newly diagnosed liver cancer worldwide with a mortality of 695,900 cases each year. More than 80% of hepatocellular carcinoma (HCC) is discovered in sub-Saharan Africa or Asia, while the Western countries have a lower incidence. For example, the annual incidence rate of HCC in the United States is (1.5–4.9)/100,000 people. And according to the prediction by American Cancer Society, the new cases of liver cancer would reach 28,720, with 20,550 deaths in 2013, but it did not belong to the top ten malignant tumors with high incidence. In our country, the annual incidence of liver cancer was 25.7/100,000, and the mortality rate was 23.7/100,000, ranking the third and second the incidence and mortality of malignant tumor, respectively. Among 40,656 cases of hepatobiliary tumor diagnosed in the Department of Pathology, Eastern Hepatobiliary Surgery Hospital (EHBH),the Second Military Medical University during the past 30 years, malignant hepatic tumors account for 80% of all the cases, and the top two are HCC (86%) and intrahepatic cholangiocarcinoma (ICC, 8%). The number of surgical cases of both cancers is still increasing (Fig. 7.1), suggesting the high incidence of liver cancers in our country [1–3]
Fig. 7.1
The proportion of surgical resected HCC/ICC
in the EHBH over the 30 years
Citation Ramadan O. Outcomes of patients with nasopharyngeal plasmacytoma: A systematic review. Otolaryngol Open J. ABSTRACT Background: This study reviews the published literature related to nasopharyngeal plasmacy-toma. Clinical presentation, demographics, treatment, and outcomes of this uncommon disease have been reported. Methods: A systematic review of studies on nasopharyngeal plasmacytoma from 1935 to 2016 was conducted. A PubMed database search was performed for articles related to this condition along with the bibliographies of those selected articles. Articles were examined for patient data that reported the disease outcome. Results: Fifty-eight journal articles were included in this analysis, comprising of a total of 114 studies indicative of a predilection for cases particularly in men with a mean age of 55 years (sixth decade of life) characterized with symptoms such as nasal obstruction, epistaxis and neck lymphadenopathy. Radiotherapy was the most common treatment modality, followed by a combination of surgery and radiotherapy. Most of the patients were alive with no evidence of the disease after the median follow-up of 59 months. Of the 3 most common treatment modalities , a combination of radiotherapy and surgery had the most favorable outcome for a majority of patients. The existing condition for 8 patients (7%) deteriorated leading to the development of multiple myeloma. Conclusion: This review contains a large pool of information about nasopharyngeal plasma-cytoma patients examined to date suggesting that aggressive radiotherapy is the most common treatment modality for this condition. Of the 3 most common treatment modalities, a combination of surgery and radiotherapy was shown to have the best survival outcomes.
Myeloid, plasma cell, and lymphoblastic neoplasms are expected findings in bone marrow but are much less commonly diagnosed as primary processes in lymph nodes. The objective of this review is to aid pathologists in recognizing common hematopoietic neoplasms in the unusual setting of initial presentation in lymph nodes. Review of historical background and evolution of testing strategies is presented in order to improve understanding of the need for accurate diagnosis and classification using current nomenclature. The review is based on peer reviewed literature and the personal experience of the authors. The University of Minnesota Medical Center, Fairview provides lymph node diagnostic consultation services for its busy oncology and therapeutic hematopoietic cell transplant divisions serving patients from around the globe. Although readily recognizable when they present in bone marrow, myeloid leukemia in the form of myeloid sarcoma, plasmacytoma, and lymphoblastic lymphoma can create diagnostic and classification challenges when they present as primary lymph node pathologies. Utilization of all diagnostic tools may be necessary to ensure accurate and reproducible diagnoses.
About 3–5 % of patients with plasma cell dyscrasias present with either a single bone lesion, or less commonly, a soft tissue mass made up of monoclonal plasma cells without evidence of bone marrow involvement or end-organ damage known as a solitary plasmacytoma (SP). SP of bone mostly occurs in axial skeleton, while it most often affects the head and neck region in case of extramedullary tumors. Their median age is 55 years and mostly present with bony pain, spinal cord, or nerve root compression. A whole body (WB) or spine and pelvic magnetic resonance imaging (MRI) scan or WB fluorodeoxyglucose–positron emission tomography (FDG–PET) scan should be included in staging of these patients. The standard of care for SP is radiotherapy (RT) given with curative intent. Surgery may be required for patients with retropulsed bone, structural instability of the bone, or rapidly progressive neurological symptoms from spinal cord compression. The role of adjuvant RT after complete surgical resection, adjuvant chemotherapy, or adjuvant bisphosphonates is not well defined and hence not recommended.
Solitary plasmacytoma (SP) is the clinical condition characterized by the localized proliferation of clonal plasma cells. The definition of SP has been evolving as a result of improvement in imaging technology as well as the availability of more sensitive techniques that can detect small populations of clonal plasma cells in the bone marrow. Patients with SP do not have other lytic or sclerotic bone lesions or soft tissue masses, hypercalcemia, renal insufficiency, or anemia and no involvement of the bone marrow by clonal plasma cells (Table 16.1) [1–3]. Some series have included patients with two bone lesions and less than 5 or 10 % clonal plasma cells in the bone marrow [4–10]. The presence of a monoclonal protein in the serum or urine or the presence of elevated immunoglobulin free light chain (FLC) does not exclude the diagnosis. On the contrary, such biomarkers may provide important prognostic information and may guide management. SP is further divided into two entities: solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMP) where the plasma cell clone generally arises from lymphoid tissues away from the bone marrow microenvironment that normally hosts these cells. SP is quite uncommon and constitutes less than 5 % of all plasma cell neoplasms [11]. Out of 45,366 patients with a plasma cell proliferative disorder seen at Mayo Clinic, Rochester, MN, between 1960 and 2011, 883 patients were diagnosed with SP (2 %). SPB is more common than EMP by a ratio of at least 2:1 [2, 12], although the comprehensive literature review by Alexiou et al. suggests a ratio closer to 5:1 [13]. SP is more common in males (~70 %) and the median age at diagnosis varies from 55 to 60 years, depending on the study [2, 5, 7, 8, 14, 15]. Almost a third of patients are below 50 years of age at the time of diagnosis. Thus, patients diagnosed with SP are significantly younger than those diagnosed with multiple myeloma.
Plasma cell malignancies are radioresponsive. Radiation is used as primary or adjunctive therapy of plasma cell disorders (PCDs). Palliation of symptoms is the commonest indication for radiation to the skeletal and soft tissue myelomatous lesions. Other indications include pretransplant conditioning, myeloma-related cord compression, or impending pathological fractures. Radiation is also utilized in the management of solitary osseous and extramedullary plasmacytomas, POEMS syndrome with a limited number of lesions, localized AL amyloidosis and rarely, Waldenström’s macroglobulinemia. Anecdotal reports outlining successful use of radiation with novel anti-myeloma agents exist. This chapter focuses on conventional radiation and chemo-radiation-based management strategies in PCDs.
Plasma cell neoplasms comprised 22% of the mature B-cell neoplasms in the Surveillance, Epidemiology, and End Results program (SEER) of the United States [26]. The majority of these are multiple myeloma, a generalized malignancy of terminally differentiated B-cells, with localized solitary plasmacytoma accounting for less than 6% of cases. This chapter will chiefly address the management of solitary plasmacytomas, as the treatment of multiple myeloma is mainly with systemic chemotherapy and the role of local therapy is restricted for palliation of symptoms. The recommended tests for the work up of plasmacytoma are to rule out the presence of systemic myeloma, as outlined in Table 1. A diagnosis of solitary plasmacytoma is made when all the following criteria are satisfied [10]: a histologically confirmed single lesion with negative skeletal imaging outside the primary site, normal bone marrow biopsy (less than 10% monoclonal plasma cells), and no myeloma-related organ dysfunction. The median age at diagnosis of solitary plasmacytoma is 55-65 years, on average about 10 years younger than patients with multiple myeloma [28, 32, 35]. Males are affected more often (male:female ratio 2:1) [28]. A monoclonal protein (M-protein) is present in 30-75% of cases (especially for a bone presentation), usually minimally elevated (IgG < 35 g/L, IgA < 20 g/L, and urine monoclonal kappa or lambda < 1.0 g/24 h) [10, 37]. Some patients with suspected solitary plasmacytoma of bone will be upstaged following magnetic resonance imaging (MRI) detection of multiple vertebral lesions or bone marrow disease [21, 23, 36], or positivity on 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) [29].
A paucity of data exists concerning the presentation, natural course and outcome of extramedullary plasmcytoma (EMP). It is difficult to determine the optimal treatment strategy and prognostic factors for EMP. We present an additional case of laryngeal EMP and systemic review relevant reports in the English and Chinese literature. We found, to our knowledge, 147 cases in larynx in the English-language literature and Chinese-literature. The most common treatment modality was radiotherapy alone. The mean survival duration was ~184 months, and the 5- and 10- year survival rates were 76.1% and 67.4%, respectively. The univariate analysis suggested that progression to multiple myeloma and amyloid deposits may be poor prognostic factors. The multivariate analysis suggested that only progression to multiple myeloma may be a poor prognostic factor. Laryngeal EMP is uncommon. Progression to multiple myeloma may be a poor prognostic factor.
Extramedullary plasmacytoma (EPM) is a plasma cell tumor arising outside of the bone marrow. Solitary EMP is an uncommon neoplasm and rarely occurs in the retroperitoneum and lacks distinctive clinical manifestations. We report a 26-year-old man with a solitary EMP in the retroperitoneum and discuss its clinical features, diagnosis and treatment.
The clinical features and response to therapy of 32 Chinese patients with localised plasmacytoma are presented, and a comparison between extramedullary plasmacytoma (EMP) and solitary plasmacytoma of bone (SPB) is made. Twenty-two patients had SPB and ten had EMP, accounting for 9% of all of our plasma cell neoplasms. Both groups had a male predominance with a median age of 54 years for SPB and 63 years for EMP. The common sites of SPB included vertebral bodies (15) and the skull (4). Most EMPs occurred in the oronasopharynx (6) and paranasal sinuses (2). An M-protein was detected in eight patients with SPB and in six with EMP. Seventeen patients with SPB and seven with EMP received radiation therapy, and all achieved initial local control. The pattern of failure in 22 patients with SPB manifested as local recurrence in two, multiple bone metastases without bone marrow plasmacytosis in two, multiple EMP progression in two, and development of multiple myeloma (MM) in one. There were two local recurrences, one further solitary bone involvement and one MM conversion in the EMP group. Local recurrence or dissemination was associated with the appearance of M-protein or an increase in the M-protein level in both groups. There was no significant difference in M-protein status or incidence and patterns of failure between the two groups. Patients with EMP had a more favourable overall survival than those with SPB (P = 0.03). The 5 year disease-free survival rate was 79% for EMP and 58% for SPB (P = 0.53). Patients aged less than 60 years had a better overall survival in the SPB group, but location of tumour, presence of M-protein, radiation dose and chemotherapy did not influence prognosis in either group. Our results indicate that adequate local therapy can result in long-term survival with a low frequency of MM progression for patients with localised plasmacytomas, and both EMP and SPB appear to be similar in terms of frequency and patterns of failure.
Extramedullary plasmacytomas (EMP) of head and neck are rare tumours. Between 1972 and 1993, 25 cases of EMP of head and neck were seen at our institute. The clinical and pathological features and response to treatment are presented. At initial presentation, 23 (92%) patients presented with disease confined to a single extramedullary site only and two patients had in addition clinical involvement of cervical lymph nodes. All except these two patients received radiotherapy to the primary site only as initial treatment. Initial primary control of local disease was obtained in 16 of 24 (67%) patients treated with radical intent. With salvage treatment of further radiotherapy and/or chemotherapy, local disease control was achieved in 21 of 24 (88%) patients. One patient was treated with palliative intent. Conversion to multiple myeloma was seen in two patients (8%). Pathologically, the tumours were classified into low, intermediate and high grade, which correlated closely with outcome. This classification has been used for the first time in extramedullary plasmacytomas and is based on the multiple myeloma grading criteria devised by Bartl et al (1987). Fifteen of eighteen (83%) low-grade tumours and only one of six (17%) intermediate- and high-grade tumours were locally controlled after primary radiotherapy. This is statistically significant for local control (P= 0.0019) but not for overall survival (P= 0.12). The median survival and 5-year overall survival is 68 months and 58.9% respectively. We recommend consideration of adjuvant chemotherapy in patients with higher grade disease.
Images
Figure 1Figure 2Figure 3Figure 4Figure 5Figure 6
Very little information is available regarding the diagnostic utility of positron emission tomography with [(18)F]fluorodeoxyglucose (FDG PET) in multiple myeloma. Our objective was to further define the role of FDG PET in the clinical assessment of patients with multiple myeloma.
Nine whole-body PET scans (45 min after intravenous administration of 370-555 MBq FDG) were performed in six patients (age 38-62 years, 5 males) with multiple myeloma for evaluation of the extent of disease at the time of initial diagnosis (n=3) and for assessment of therapy response (n=3). Three patients had PET scans both before and after therapy. Prior treatments included chemoradiation therapy (n=2) and chemotherapy with autologous bone marrow transplantation (n=1). Correlative imaging data were available in all patients and included skeletal radiographic survey (n=6), bone scan (n=3), and spinal CT or MRI (n=4), and were all obtained within 3 months of the PET study. Validation was by clinical or imaging follow-up.
In three patients with both pre- and post-therapy PET scans, PET demonstrated a favorable treatment response, by showing a decline in lesion metabolic activity (n=1), or progression of disease, by showing development of new lesions or higher lesion glucose metabolism (n=2), concordant with the clinical evaluation, while the other imaging studies showed no discernible interval changes. PET detected multiple hypermetabolic lesions in one patient with a negative bone scan and concordant positive skeletal radiographic survey. Bone scans underestimated the extent of disease in two other patients in comparison with PET. PET also detected a few early marrow lesions with subtle radiographic changes while all radiographically aggressive lytic lesions corresponded to intense hypermetabolism on PET.
PET can detect early marrow involvement of multiple myeloma and is useful in assessing the extent of active disease at the time of initial presentation and in evaluating treatment response.
Extramedullary plasmacytomas are solitary tumors consisting of neoplastic plasma cell proliferations that occur in locations other than bone. On initial presentation they must be differentiated from multiple myeloma. This may prove to be difficult because a varying percentage may be associated at a later date with the development of multiple myeloma. Solitary extramedullary plasmacytomas represent up to 4% of nonepithelial lesions of the upper respiratory tract. From 1970 to 1990, at West Virginia University Hospitals, seven patients with solitary extramedullary plasmacytoma were identified. In four of these patients the tumor was located in the head and neck, with one tumor located in each of the following sites: temporoparietal scalp, maxillary sinus, nasopharynx, and cervical region. One patient had extensive destruction of the temporal bone, with extension intracranially to the middle cranial fossa. No patient had multiple myeloma, nor did any develop. Diagnosis was based on a combination of histology along with special immunoperoxidase staining for Ig lambda and kappa light chains. This will be demonstrated and discussed. Treatment consisted of radiotherapy in three cases, with doses ranging from 3175 to 6000 rad. One patient, treated with surgical excision, experienced a relapse at a distant site 6 years later. All patients have maintained local control and have been followed for a minimum of 1 1/2 years, with an average of 3 years. We describe our experience with these tumors and present a pertinent review of the literature. While these tumors may present as aggressive locally destructive lesions, their management should be as organ-sparing as possible because excellent control can be achieved in the majority of cases. Patients should be followed for life because multiple myeloma may develop in a significant number.
A retrospective review is reported of 128 patients presenting with multiple myeloma and 16 patients presenting with solitary plasmacytoma. Ninety-one percent of 116 evaluable patients treated for palliation of painful bone disease received some degree of subjective pain relief. The radiation dose most frequently prescribed was between 1500 and 2000 rad. Of the 278 ports treated, only 17 (6.1%) were re-treated to the same area at a later date. There was no increase in incidence of re-treatment with lower radiation doses. Ten of the 13 patients treated for a solitary plasmacytoma with a minimum follow-up period of three years have local tumor control. The median survival in the solitary plasmacytomas is five and one-half years. Data from the literature on 27 additional solitary plasmacytomas combined with our data suggest an improved local control and a decrease in dissemination with doses greater than 5000 rad. It is concluded that low doses of radiation are usually adequate to treat painful bone lesions of multiple myeloma and doses of 5000 to 6500 rad in six to seven weeks are recommended for solitary plasmacytomas.
From this study I suggest that extramedullary plasmacytoma (EMP) shows several important differences from myelomatosis and solitary myeloma of bone (SMB) which can be summarized as follows: 1. A marked preference for the primary tumor to present in a particular site, namely the upper air passages. 2. A high incidence of metastatic spread to soft tissues. 3. Spread to bone occurs frequently but shows no preference for bones containing active hematopoietic tissue and widespread bone-marrow involvement occurs only occasionally. 4. Prolonged survival may be achieved with therapy for local disease. 5. Vigorous treatment for disseminated disease can be given and may result in longer remissions than those usually seen in myelomatosis. Healing of bone lesions has been observed on several occasions. It is concluded also that SMB constitutes a rather unusual presentation of myelomatosis but is essentially the same disease process.
The clinical features and response to treatment of 35 patients (pts) with extramedullary plasmacytoma (EMP) were retrospectively analysed. The median age at diagnosis was 49 years (28-72). Twenty-two pts (63%) had stage I disease (localized to the primary site) 12 of whom (34%) had stage I-E (locally extended). Three pts (9%) had stage II (regional lymph nodes involved) and 10 (29%) stage III (disseminated disease). In locoregional disease (stages I, I-E, II) complete local control was achieved in 22 of 25 pts (88%), while in diffuse disease (stage III) complete remission (CR) was obtained in 5 of 10 pts (50%) (p = 0.05). In 9 of 18 pts treated with surgery, local control was achieved, and in 8 of the 9 patients with incomplete resection local control was obtained with additional radiation and/or chemotherapy. In 8 (66%) of the 12 pts treated with radiation complete local control was achieved. In 11 (58%) of 19 pts evaluable for initial chemotherapy CR was obtained. Three of these pts were treated with chemotherapy only and were alive and disease-free after a minimum follow-up of 8 years. The median time to relapse in local disease was 48 months versus 13 in disseminated disease. For pts with local disease the median survival time was 114 months and for disseminated disease 16 months (p = 0.0000). We conclude that in stage I chemotherapy is curative per se. In local stages (I, I-E and II) adjuvant chemotherapy should be considered, while in stage III only palliative therapy is feasible.
Between 1960 and 1985, 30 patients with solitary plasmacytomas were treated with radiotherapy at the University of Iowa: 13 patients with extramedullary plasmacytomas (EMP) and 17 with solitary plasmacytomas of bone (SPB). The local control rates were 92% for patients with EMP and 88% for those with SPB. Two of nine patients (22%) with EMP treated to the primary tumor only developed regional lymph node metastasis, indicating the need for elective irradiation of this area. The most common pattern of failure in both groups was progression to multiple myeloma. This occurred in 23% of the patients with EMP and 53% of those with SPB. The time course of progression to multiple myeloma differed for the two groups. All of those who progressed to multiple myeloma in the EMP group did so within 2 years, whereas a significant number of those in the SPB group progressed more than 5 years after initial therapy. None of five patients who received adjuvant chemotherapy in the SPB group progressed to multiple myeloma, compared to 75% (9/12) of the patients who did not receive chemotherapy.
A retrospective review is reported of 128 patients presenting with multiple myeloma and 16 patients presenting with solitary plasmacytoma. Ninety-one percent of 116 evaluable patients treated for palliation of painful bone disease received some degree of subjective pain relief. The radiation dose most frequently prescribed was between 1500 and 2000 rad. Of the 278 ports treated, only 17 (6.1%) were re-treated to the same area at a later date. There was no increase in incidence of re-treatment with lower radiation doses. Ten of the 13 patients treated for a solitary plasmacytoma with a minumum follow-up period of three years have local tumor control. The median survival in the solitary plasmacytomas is five and one-half years. Data from the literature on 27 additional solitary plasmacytomas combined with our data suggest an improved local control and a decrease in dissemination with doses greater than 5000 rad. It is concluded that low doses of radiation are usually adequate to treat painful bone lesions of multiple myeloma and doses of 5000-6500 rad in six to seven weeks are recommended for solitary plasmacytomas.
A prospective CT study of seventeen patients with metastatic cervical adenopathy from tumors at an unknown primary site, believed most likely to be in the upper aerodigestive tract, was performed. All patients had normal endoscopic examinations performed by an otolaryngologist before the CT study. CT suggested a primary site in ten cases, of which four were confirmed by surgery or biopsy. In four cases, CT did not identify a suspicious site and no primary site was identified during triple endoscopy, sometimes with blind biopsies. CT was able to suggest correct causes other than carcinoma in three cases and gave useful information about the extent of nodal disease. CT should be used as part of the routine evaluation of patients with this clinical problem. A diagnostic algorithm is suggested in which CT is integrated with modern endoscopic and aspiration cytologic techniques.
Patients with solitary osseous plasmacytomas (SOP) differ from those with extramedullary plasmacytomas (EMP) in that they are younger and the proportion of males is smaller. The median survival of the two groups is similar: 86.4 mo for SOP, and 100.8 mo for EMP. Progression-free survival, however, is much better for EMP. Only five EMP patients have progressed following initial radiation therapy: one developed a single bony lesion, two progressed to multiple myeloma, and two developed multiple EMP. Thus, 71% of EMP patients are progression free at 10 yr, and most deaths do not result from plasma cell neoplasia. In contrast, 13 SOP patients have progressed to develop additional osteolytic lesions, so that only 16% of SOP patients are progression free at 10 yr; death resulted from progression to multiple myeloma in most of these patients. In EMP patients the occurrence of involved lymph nodes at the time of diagnosis in seven, and initial relapse in regional nodes in three, suggest that consideration should be given to including regional lymph nodes in the radiation fields used to treat these patients.
Between 1962 and 1978, 15 patients presenting with a solitary plasmacytoma were treated with curative intent by radiotherapy alone at the University of Florida. Criteria for admission to this study were: 1) a biopsy-proven, apparently solitary focus of plasmacytoma; 2) bone marrow biopsy showing less than 10% plasma cells; and 3) no evidence of disseminated disease. In 9 patients the primary site was osseous and in 6 patients extramedullary; 5 of the 6 extramedullary lesions were located in the upper respiratory passages. This study presents a detailed analysis of the time-dose relationship required for local control, based on a study of our own patients and a review of the literature.
The purpose of this study is to report the results of megavoltage irradiation in 23 previously unreported cases of extramedullary plasmacytoma of the head and neck. It has been found that 3500 cGy (rad) in three weeks provides good local control of disease with minimal morbidity and a significant proportion do not go on to multiple myeloma. Prognostic factors of significance with respect to subsequent development of multiple myeloma include site and presence or absence of bone destruction. The presence or absence of an M protein peak appears to be of no significance.
Within plasma cell disorders, solitary myeloma is rare as compared with multiple myeloma. In order to evaluate their relationship, the clinical findings for 114 patients with solitary myeloma were compared with those for 70 patients having classic multiple myeloma. The period of follow up ranged from a few weeks to twenty-four years, and 68.5% of those with solitary myeloma alive at ten years. Although only 23% of patients with solitary myeloma had local or widespread recurrence after two years, at ten years 85% had experienced disease progression. Comparison of the 85% with disease progression with patients with multiple myeloma revealed that solitary myeloma occurred at a younger age (mean 52.1 years), more frequently in men (60.5%), less commonly with initial spinal involvement (61.8%), but more commonly with neurologic problems associated with spinal involvement (25%), and that much more commonly, no monoclonal component was detectable in serum and/or urine at the time of initial diagnosis (82.5%). There were only two significant differences between those patients with (85%) and without (15%) progression at ten years; the patients were younger (mean, 45.7 years) and spinal involvement (26.7%). However, was less common among patients without progression, and component monoclonal always disappeared following treatment with surgery and/or radiation therapy. It is thus concluded that solitary myeloma and multiple myeloma are clearly different clinical entities; however, most patients with solitary myeloma do eventually have multiple myeloma.
One hundred and forty-four patients with diagnosis of carcinoma involving cervical lymph nodes from an unknown primary site were seen at the Princess Margaret Hospital, Toronto between 1970 and 1986. This paper addresses the outcome of two treatment approaches using radiation treatment as potentially curative therapy. The treatment approaches compared in a retrospective analysis are radiation to the involved node regions alone (85 cases), and radiation to both the nodes and to potential primary sites in the head and neck (59 cases). The overall 5-year survival rate of the entire group of 144 cases was 41%. There was a trend to increased survival in favour of the group receiving radiation to the nodes and potential primary sites (p = 0.07), however, when differences in extent of nodal involvement were considered and adjusted for using the Cox Proportional Hazard Model, no difference in survival, or cause-specific survival was found between the two treatment groups (p = 0.18 and 0.22, respectively). A total of seven head and neck primary cancers were subsequently discovered, six in the group receiving radiation only to the nodes and one in the group receiving radiation to the nodes and primary sites. The results of this retrospective analysis need to be interpreted with caution but suggest that, for some patients with this diagnosis, radiation to the involved node region alone is adequate. A discussion of the literature is presented with attention to the effect of extent of treatment on outcome.
Extramedullary plasmacytomas are solitary tumors consisting of neoplastic plasma cell proliferations that occur in locations other than bone. On initial presentation they must be differentiated from multiple myeloma. This may prove to be difficult because a varying percentage may be associated at a later date with the development of multiple myeloma. Solitary extramedullary plasmacytomas represent up to 4% of nonepithelial lesions of the upper respiratory tract. From 1970 to 1990, at West Virginia University Hospitals, seven patients with solitary extramedullary plasmacytoma were identified. In four of these patients the tumor was located in the head and neck, with one tumor located in each of the following sites: temporoparietal scalp, maxillary sinus, nasopharynx, and cervical region. One patient had extensive destruction of the temporal bone, with extension intracranially to the middle cranial fossa. No patient had multiple myeloma, nor did any develop. Diagnosis was based on a combination of histology along with special immunoperoxidase staining for Ig lambda and kappa light chains. This will be demonstrated and discussed. Treatment consisted of radiotherapy in three cases, with doses ranging from 3175 to 6000 rad. One patient, treated with surgical excision, experienced a relapse at a distant site 6 years later. All patients have maintained local control and have been followed for a minimum of 1 1/2 years, with an average of 3 years. We describe our experience with these tumors and present a pertinent review of the literature. While these tumors may present as aggressive locally destructive lesions, their management should be as organ-sparing as possible because excellent control can be achieved in the majority of cases.(ABSTRACT TRUNCATED AT 250 WORDS)
To evaluate 2-[fluorine-18]fluoro-2-deoxy-D-glucose (FDG) single photon emission computed tomography (SPECT) in the detection of clinically occult primary tumors of the upper aerodigestive tract.
Eighteen patients with histologically proved cervical nodal metastasis from squamous cell carcinoma (SCC) underwent FDG SPECT; 17 also underwent computed tomography (CT) and one underwent magnetic resonance (MR) imaging. All 18 patients underwent direct panendoscopy and biopsy. Biopsy and imaging results were correlated.
Among 11 histologically proved primary tumors, FDG SPECT depicted nine tumors; CT depicted four lesions. Five patients had abnormal FDG uptake with negative biopsy results. Among five patients with normal SPECT findings, biopsy results were positive in two and negative in three. FDG SPECT at the initial reading had sensitivity of 81%, specificity of 38%, positive predictive value of 64%, and negative predictive value of 60% for detection of occult primary tumors.
FDG SPECT guidance of endoscopic biopsies in patients with occult primary SCCs of the head and neck has the potential to yield a higher rate of positive biopsy results than that traditionally expected from "blind" endoscopic procedures with "random" or "speculative" biopsy. FDG SPECT and CT are likely to remain complementary studies for the detection of occult primary tumors of the head and neck.
Solitary plasmacytoma of bone (SPB) and extramedullary plasmacytoma (EMP) are rare. High local control rates are reported with radiotherapy, although the optimal dose and extent of radiotherapy portals remains controversial. Between 1983 and 1993, 30 patients with solitary plasmacytoma were seen at the Regional Cancer Centre, Trivandrum, India. 23 patients had SPB and seven EMP. The mean age was 52 years and the male to female ratio 3.2:1. Diagnosis of SPB was confirmed by biopsy in 16 patients and tumour excision in seven. 20 patients received megavoltage radiotherapy to the bone lesion with limited margins, and one received chemotherapy. Two patients who underwent complete tumour excision received no further treatment. All seven patients with EMP received megavoltage radiotherapy, four following biopsy and three after tumour excision. Local control was achieved in all patients with SPB. Nine progressed to multiple myeloma and one developed a solitary plasmacytoma in another bone. Six patients with EMP achieved local control. Three later progressed to multiple myeloma and one had local relapse. Median time to relapse was 28 months in SPB and 30 months in EMP. 5-year overall survival rates were 82% and 57% for patients with SPB and EMP, respectively. The corresponding progression free survival rates were 55% and 50%, respectively. Age, sex, site of tumour, serum M protein and haemoglobin levels did not significantly influence progression free survival. The extent of surgery, radiotherapy dose or time to relapse were not significant prognostic factors. Radiotherapy appears to be an effective modality of treatment of solitary plasmacytoma. No dose-response relationship is observed, and high local control rates are achieved with limited portals. Progression to multiple myeloma is the commonest pattern of failure, although no prognostic factors for progression are identified. The role of chemotherapy in preventing disease progression needs further evaluation.
In patients with cervical node metastases from an unknown primary malignancy, there is unresolved controversy regarding the utility of elective irradiation of putative pharyngeal primary sites as part of the management plan. We analysed the experience of the Peter MacCallum Cancer Institute to assess the risk of withholding mucosal irradiation in relation to the diagnostic algorithm used to exclude a primary lesion at the time of initial presentation. Between 1983 and 1992, 69 patients were seen with metastatic squamous or undifferentiated carcinoma in cervical nodes from an unknown primary site. Neck nodal stage was NX or N1 13%; N2 52%; N3 35%. Nodal disease was bilateral in 12% of patients. Investigations included examination under anaesthesia, with or without random biopsies, in 84%, and CT scanning of the head and neck in 55%. Treatment was by surgery alone in four patients, by radiotherapy alone in 23, and by combined modalities in 40. Two patients received no treatment. Seventeen were treated with palliative intent. The radiotherapy fields provided comprehensive coverage of the pharynx in only eight patients and partial coverage in five. The estimated overall 5-year survival was 36%. Eleven primary tumours were detected between 7 months and 7 years after the initial treatment, of which nine were in head and neck sites. This yielded an estimated incidence of 30% at 10 years, which is similar to the risk of the development of a second primary after the successful treatment of a known head and neck cancer. Only three patients (none of whom had a CT scan as part of their initial evaluation) manifested a primary in an unirradiated pharyngeal site within 2 years of treatment. As the accuracy of imaging improves, the risk of missing an occult primary lesion will decrease further. We conclude that the use of standardized diagnostic investigations incorporating modern imaging substantially eliminates the indication for comprehensive elective mucosal irradiation with its consequent morbidity. The overriding priority in patients who present with advanced neck disease is to secure regional control.
Plasmacytomas are rare tumors that often appear in the head and neck region and are characterized by a monoclonal proliferation of plasma cells. On both clinical presentation and pathologic examination these tumors may be confused with more common tumors of the head and neck. The purpose of this article is to review our experience with these rare neoplasms, with emphasis on clinical, pathologic, and therapeutic features. On retrospective chart review, we identified 20 patients with the diagnosis of plasmacytoma of the head and neck region at the Cleveland Clinic Foundation between 1976 and 1993. Records were reviewed with regard to initial symptoms, location of the neoplasm, diagnostic evaluation, treatment modalities, and survival. Of the 20 cases we identified, the tumor arose in the sinonasal/nasopharyngeal region in 11 (55%). Two cases (10%) represented medullary plasmacytomas, arising in the clavicle and presenting as supraclavicular masses. The mean follow-up was 60.2 months (range 6 to 131 months). In 15 of the 20 cases, immunohistochemistry staining for immunoglobulin light chain production was conducted. One of the two cases (50%) classified as medullary plasmacytoma demonstrated conversion to multiple myeloma, whereas only 2 of 18 cases of extramedullary plasmacytoma (11%) converted to multiple myeloma. The primary modality of treatment was radiation therapy with typical doses of 4500 to 6000 cGy. Kaplan-Meier survival estimates demonstrated 95% survival at 1 year, 82% survival at 5 years, and 10-year estimated survival of 72%. Plasmacytomas of the head and neck region are rare and on initial evaluation must be distinguished from multiple myeloma. The diagnostic evaluation includes appropriate radiologic and pathologic studies including immunohistochemistry. Despite the typical presentation as a locally destructive tumor, plasmacytomas are highly radiosensitive, and 70% to 80% survival may be obtained with the use of radiotherapy. Patients with plasmacytomas require long-term follow-up to detect conversion to multiple myeloma.
Solitary extramedullary plasmacytoma (EMP) represents a rare category of malignant disease on which there are limited data in regard to diagnosis, staging and natural history. This study attempted to clarify the clinical course of solitary extramedullary plasmacytoma after radiation or surgical therapy given with curative intent.
The diagnosis was based on a mass of clonal plasma cells separate from bone or bone marrow without evidence of occult disease elsewhere. Between 1963 and 1996, 22 previously untreated patients with an EMP were diagnosed. Disease presented in the head or neck in 86%, usually in the nasal cavity (NC) or maxillary sinus (MS), and in these areas local bone destruction was found in 10 of 11 patients. Among all patients, serum myeloma protein was present in three patients (14%) and Bence Jones protein alone was found in two patients (9%). Radiation therapy was the sole treatment for 18 of 22 patients, and the median radiotherapy dose was 50 Gy (range, 40-60 Gy); five of seven patients with an EMP of oral cavity (OC), oropharynx (OP), nasopharynx (NP), parotid or larynx also received elective neck irradiation. Two patients underwent surgery plus postoperative irradiation of a plasmacytoma of the sigmoid colon or pleura, and two patients had resection alone of a plasmacytoma of the colon or cervical lymph node.
Local control was achieved in 21 of 22 patients (95%), and disease never recurred in regional nodes. Disappearance of myeloma protein occurred in three of five patients with an evaluable abnormality. Multiple myeloma developed in seven patients (32%), all within 5 years. The 5-year rate of freedom from progression to multiple myeloma was 56% and the median survival was 9.5 years.
Radiation therapy achieved excellent locoregional control of EMP with an approximate cure fraction of 50%.
This prospective study was undertaken to investigate the appearance of multiple myeloma on fluorine-18 fluorodeoxyglucose positron emission tomography (FDG-PET). Furthermore, the accuracy of FDG-PET in detecting myeloma lesions and its influence on patient management were evaluated. Forty-three patients with known multiple myeloma (n=28) or solitary plasmacytoma (n=15) underwent FDG-PET. The results of routinely performed radiographs and of scans obtained using all available imaging modalities (MRI, CT), as well as the clinical course, were used for verification of detected lesions. Focally increased tracer uptake was observed in 38 of 41 known osteolytic bone lesions (sensitivity 92.7%) in 23 patients. In addition, 71 further bone lesions which were negative on radiographs were detected in 14 patients. Twenty-six (36.6%) of these lesions could be confirmed in ten patients. As a result of FDG-PET imaging, clinical management was influenced in five (14.0%) patients. The positive predictive value for active disease was 100% in patients with focal or mixed focal/diffuse skeletal FDG uptake and 75% in patients with diffuse bone marrow uptake. Depending on the interpretation of the PET scans in patients with diffuse bone marrow uptake, the sensitivity ranged from 83.8% to 91.9% and the specificity from 83.3% to 100%. FDG-PET thus proved highly accurate in detecting multiple myeloma, and revealed a greater extent of disease than routine radiographs in 14 of 23 (60.9%) patients who had osteolytic bone lesions. FDG-PET might contribute to the initial staging of solitary plasmacytoma.
To review the histories of extramedullary plasmacytoma patients diagnosed in Slovenia between 1969 and 1999, to determine the relationship between radiotherapy (XRT) dose and local tumor control, and to clarify the role of elective nodal XRT and the prognostic value of Bartl's histologic grading criteria (originally devised for multiple myeloma [MM]).
The database of the Cancer Registry of Slovenia was used for the identification of patients. The inclusion criteria were as follows: bone marrow biopsy showing less than 10% plasma cells, normal skeletal survey, and immunohistochemically determined tumor monoclonality. Simulation/portal films were reviewed to assess the extent of elective nodal XRT.
Twenty-six patients with 31 tumors fulfilled the inclusion criteria. In 4 patients, nine metachronously appearing solitary tumors were diagnosed. The head-and-neck region and other body sites were the sites of origin of primary tumors in 84% and 16% of patients, respectively, whereas in the two regions, regional disease was seen in 15% and 60% of patients, respectively. Therapy was as follows: XRT, 12 patients; surgery and postoperative XRT, 15 patients; and surgery, 4 patients. Ultimate local and regional control rates were 90% and 97%, respectively, and MM developed in 2 (8%) patients. The 10-year disease-specific and overall survival rates were 87% and 61%, respectively. The analysis of the dose-effect relationship showed that more conservative treatment is justified: for macroscopic disease, 40-50 Gy (2 Gy/day), adjusted to the bulk of disease; for microscopic disease, 36-40 Gy; after R0 surgery, no XRT is required, but close observation is needed. No attempts should be made to treat uninvolved nodal regions. Using Bartl's histologic grading criteria, trends were detected in patients with higher tumor grades: regional lymph node involvement (p = 0.04) and shorter disease-specific survival (p = 0.08).
Extramedullary plasmacytoma is a highly curable disease when XRT is used with or without previous surgery. The rate of conversion to MM is low. Moderate-dose XRT using limited fields is recommended. The prognostic value of Bartl's grading system needs further evaluation.