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a Overall survival and b progression-free survival for patients with localized sinonasal diffuse large B cell lymphoma (SN-DLBCL, n = 47) and sinonasal extranodal NK/T cell lymphoma (SN-ENKTL, n = 211)
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The difference in clinical features and treatment outcomes between localized sinonasal diffuse large B cell lymphoma (SN-DLBCL) and sinonasal extranodal NK/T cell lymphoma (SN-ENKTL) is unclear. Therefore, we analyzed a total of 47 patients with localized SN-DLBCL and 211 patients with localized SN-ENKTL. The age distribution for these two subtypes...
Context in source publication
Context 1
... treatment outcomes were similar between SN- DLBCL and SN-ENKTL. The 3-year overall survival (OS) and progression-free survival (PFS) rates were 79.7 and 61.4% for SN-DLBCL and 83.6 (p = 0.707) and 70.1% (p = 0.294) for SN-ENKTL, respectively (Fig. 1). Unfavorable Eastern Cooperative Oncology Group (ECOG) performance status (PS) and failure to achieve CR were significantly associated with worse OS and PFS for SN-DLBCL patients (Additional file 2). For patients with SN-ENKTL, factors related to worse OS and PFS included unfavorable modified Ann Arbor stage and fail- ure to achieve CR ...
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Citations
... По данным литературы, наиболее распространенным экстранодальным поражением при лимфомах в обла сти головы и шеи является лимфатическое глоточное кольцо (кольцо Пирогова-Вальдейера) [14]. К прева лирующим иммуноморфологическим вариантам НХЛ, поражающим синоназальный тракт, относят ДВКЛ и экстранодальную NK / Tклеточную лимфому [15]. Сле дует отметить, что при экстранодальной NK / Tклеточной лимфоме чаще вовлекается полость носа, в то время как при ДВКЛ поражаются околоносовые пазухи [16]. ...
... В приведенном нами клиническом случае диагноз верифицирован в возрасте 63 лет. Ранняя диагностика первичной си ноназальной НХЛ крайне затруднена, поскольку по ражения, возникшие в этой области и распространя ющиеся на околоносовые пазухи или в носовую полость, наиболее часто на ранней стадии заболевания являются бессимптомными [15]. ...
Diffuse large B-cell lymphoma is the most common immunomorphological variant of lymphoma in adults. Extranodal lesions are observed in a third of patients at the disease onset. The organs most often involved are the gastrointestinal tract, testicles, bones, thyroid gland, and skin. Primary involvement of the nasal cavity and paranasal sinuses occur extremely rarely and cause diagnostic and therapeutic difficulties.
The article demonstrates a rare clinical case of newly diagnosed diffuse large B-cell lymphoma with sinonasal tract involvement. It took 6 months to verify the final diagnosis. At the moment, the induction stage of treatment for diffuse large B-cell lymphoma continues, the achieved complete metabolic response is maintained.
... In a Brazilian retrospective cohort analysis, almost 20% of cases were extranasal in origin, and, as bone marrow involvement is rare, pancytopenia should draw attention to the diagnosis of hemophagocytic syndrome. Systemic symptoms are variable and may occur, especially in advanced disease (19). Primary extranasal disease seems to confer worse outcomes, even when compared to stage III/IV nasal disease (18). ...
Extranodal NK-/T-cell lymphoma (ENKTCL) is a rare and highly aggressive malignancy with significant racial and geographic variations worldwide. In addition to the formerly “nasal-type” initial description, these lymphomas are predominantly extranodal in origin and typically cause vascular damage and tissue destruction, and although not fully understood, Epstein–Barr virus (EBV) has an important role in its pathogenesis. Initial assessment must include a hematopathology review of representative and viable tumor areas without necrosis for adequate immunohistochemistry studies, including EBV-encoded small RNA (EBER) in situ hybridization (ISH). Positron emission tomography with 18-fluorodeoxyglucose (¹⁸F-FDG-PET/CT) for accurate staging is essential, and most patients will have localized disease (IE/IIE) at diagnosis. Apart from other T-cell malignancies, the best treatment even for localized cases is combined modality therapy (chemotherapy plus radiotherapy) with non-anthracycline-based regimens. For advanced-stage disease, l-asparaginase-containing regimens have shown improved survival, but relapsed and refractory cases have very poor outcomes. Nowadays, even with a better understanding of pathogenic pathways, up-front therapy is completely based on chemotherapy and radiotherapy, and treatment-related mortality is not low. Future strategies targeting signaling pathways and immunotherapy are evolving, but we need to better identify those patients with dismal outcomes in a pre-emptive way. Given the rarity of the disease, international collaborations are urgently needed, and clinical trials are the way to change the future.
... In sinonasal lymphoma, ENKTCL was more common than DLBCL among Chinese patients [10], while sinonasal ENTKL was much rarer than sinonasal DLBCL in the US [11]. Eide et al. [12] revealed that the most common subtype of sinonasal lymphoma was DLBCL, succeeded by ENK-TCL. ...
... While radiotherapy was an independent prognostic factor only in stage I patients, and immunotherapy was independently correlated with prolonged OS in stage IV patients alone [14]. Chemotherapy followed by involvedfield radiotherapy yielded longer OS compared with chemotherapy alone for sinonasal DLBCL [10]. Another NCDBbased study which enrolled 2073 sinonasal DLBCL patients reported the OS outcomes of various therapeutic modalities in early-and late-stage patients. ...
Primary head and neck hematolymphoid neoplasms (PHNHLN) are defined as a series of hematolymphoid system-derived neoplasms which primarily emanate in head and neck region. Due to the rarity and absence of symptomatic specificity, PHNHLN is easily neglected. The objective of this study is to investigate demographics, pathological subtype distribution, anatomical location, survival outcomes and prognostic factors of PHNHLN among older patients aged ≥ 60. The individual patient information in our study was derived from Surveillance, Epidemiology and End Results database. Descriptive epidemiological methods were used to analyze the distribution of histologic subtypes and primary anatomical sites. Kaplan–Meier survival curves and log-rank test were conducted to evaluate the effect of variables on the prognosis. Cox hazard regression was conducted to identify the independent prognostic factors. The male-to-female ratio in most pathological subtypes was close to 1:1. The most common pathological subtype was diffuse large B-cell lymphoma. The most commonly involved sites outside the lymph nodes were salivary glands, especially parotid gland, followed by tonsil, thyroid gland and tongue. The prognosis of mature T- and NK-cell non-Hodgkin lymphoma (NHL) was bleaker than Hodgkin lymphoma, mature B-cell NHL and plasma cell neoplasm. Age at diagnosis, presence of second primary malignancy (SPM), pathological subtype, Ann-Arbor stage, chemotherapy and radiation were independent prognostic factors of overall survival. Our study comprehensively reported the subtype distribution, anatomical sites and survival outcomes of PHNHLN among older patients, improving understanding of this rare group of cancer entities.
... 2,38 It is most often due to malignant transformation of NK-cells rather than transformation of cytotoxic T-cells and is characterized by progressive necrotizing local destruction of bone, soft tissue, and cartilage. 39,40 SN-ENKTL is most prevalent in Asia, Mexico, and Central and South America, where it is estimated to account for 3%-10% of all lymphomas whereas there is less than 1% prevalence in western countries. [39][40][41][42][43][44][45][46][47] A recent SEER database study showed a specific incidence rate for SN-ENKTL to be 0.032 per 100,000 with an annual increase in incidence of 9%. ...
... 39,40 SN-ENKTL is most prevalent in Asia, Mexico, and Central and South America, where it is estimated to account for 3%-10% of all lymphomas whereas there is less than 1% prevalence in western countries. [39][40][41][42][43][44][45][46][47] A recent SEER database study showed a specific incidence rate for SN-ENKTL to be 0.032 per 100,000 with an annual increase in incidence of 9%. 32 There is a 3:1 male: female predominance and presentation occurs in the fifth and sixth decades of life. ...
... 5 The current most common treatment for SN-DLBCL includes multiagent chemoradiation that consists of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). 39,90,115,116 Traditionally, DLBCL has been treated with 6 cycles of R-CHOP. ...
Objective
Sinonasal lymphomas are a rare entity that commonly present with nonspecific sinonasal symptoms and are often recognized immediately. Through this review, we aim to summarize important principles in diagnosis and treatment of sinonasal lymphomas, with the goal of disseminating the current knowledge of this under‐recognized malignancy to otolaryngologists.
Methods
Systemic review using PRISMA guidelines of foundational scholarly articles, guidelines, and trials were reviewed focusing on clinical characteristics of key sinonasal lymphoma subtypes, along with available treatments in the otolaryngology, medical oncology, and radiation oncology literature.
Results
Sinonasal lymphoma are derived from clonal proliferation of lymphocytes at various stages of differentiation, of which diffuse large B‐cell lymphoma (DLBCL) and extranodal natural killer/T‐cell lymphoma (ENKTL) are the most common. Diagnosis and staging require biopsy with immunohistochemistry in conjunction with imaging and laboratory studies. Treatment is ever evolving and currently includes multi‐agent chemotherapy and/or radiation therapy.
Conclusion
Otolaryngologists may be the first to recognize sinonasal lymphoma, which requires a comprehensive workup and a multidisciplinary team for treatment. Symptoms are nonspecific and similar to many sinonasal pathologies, and it is crucial for otolaryngologists to keep a broad differential.
Level of Evidence
5
... [10] The histologic types are mainly non-Hodgkin malignant lymphoma, particularly diffuse large B-cell lymphoma (DLBCL) and natural killer (NK) T-cell lymphoma are common variants. [13] In Caucasian populations, DLBCL is common, whereas NK/T cell lymphoma predominates in South America. [12] The histological pattern of the lymphoma in a study includes 82% DLBCL and 18% NK/T cell lymphoma. ...
Background: Primary sinonasal non-Hodgkin's lymphomas (NHLs) are uncommon malignancies. The lack of specific clinical presentations often causes delayed visit to the clinician and so diagnosis is done in late with advanced stage. Objective: The objective of the study was to study the primary sinonasal NHL with respect to clinical presentations, management, and outcome. Materials and Methods: This is a retrospective study conducted in a tertiary care teaching hospital from March 2010 to April 2020. The clinical data of the patients of primary sinonasal NHL were evaluated with respect to the age, sex, clinical presentations, staging, diagnosis, treatment, and outcome. Results: The mean age of the participants was 62.45 years with male-to-female ratio of 1.5:1. All the cases of sinonasal NHL involved with paranasal sinuses and nasal cavity. Nasal obstruction was the most common symptom. All were diagnosed with large B-cell lymphoma and treated with combined chemotherapy and radiation. Five patients were died during follow-up period. Conclusion: Primary sinonasal NHLs are extremely uncommon clinical entity. The diagnosis is based on the histological examination with immunohistochemical study. Early diagnosis and prompt treatment with chemo-regimen and radiotherapy are essential to achieve an optimum outcome. Early diagnosis and treatment improves the treatment outcome.
... The therapeutic management is complex and requires surgery, chemotherapy and radiotherapy, offering a reserved prognosis [23] Other histological varieties were lymphomas, mostly histological subtype B, although we also found a case of nasal type T/NK lymphoma, the most common varieties in this location. Within the head and neck, the majority of the extranodal lymphomas emerge in the Waldeyer ring; comprised of the adenoid tonsils, Eustachian tonsils, palatal tonsils, and lingual tonsils, although other commonly involved sites include the nasal cavity and sinuses [24,25]. An important histological subtype is the adenoid cystic carcinoma (Table 3), (old cylindroma) which, although it tends to have a predilection for the head and neck, actually represents less than 2% of malignant tumours of the head and neck and 5 to 15% of paranasal sinus although it has a high capacity for local infiltration [26,27]. ...
Background:
Diagnosis of maxillary sinus pathology must include the clinical radiological study (CRS) and histopathological analysis. The aim of this study is 1) to describe the clinicopathological features of maxillary sinus lesions, obtained successively in a single medical centre over the last 10 years and 2) to determine the sensitivity and specificity for the diagnosis of malignant lesions based exclusively on the CRS.
Methods:
It is a single-centre observational retrospective clinical study on patients who attended the University Hospital Complex of Santiago de Compostela (CHUS) with sinus pathologies during the period of 2009-2019.
Results:
The sample consisted of 133 men (62.1%) and 81 women (37.9%), with an average age of 46.9 years (SD = 18.8). In terms of frequency, the most frequent pathology was the unspecified sinusitis (44.4%), followed by polyps (18.2%), malignant tumours (9.8%), inverting papilloma (7.5%), fungal sinusitis (4.7%), cysts (3.7%), benign tumours (2.3%), mucocele (2.3%) and other lesions (1.9%). Cysts and benign tumours were diagnosed earliest Vs malignant tumours (65.2 years (SD = 16.1)) were diagnosed the latest (p < 0.001). Based only on the CRS for malignancies, diagnostic indexes were 71.4% sensitivity and 97.9% specificity, with a Kappa value of 0.68 with (p < 0.001).
Conclusion:
Maxillary sinus pathology is very varied with therapeutic and prognostic repercussions. CRS is sometimes insufficient and histopathological confirmation is essential.
... Originally, ENKTL arises predominantly from natural killer cell and DLBCL arises from mature B lymphocytes. Treatment strategies for the two histologic subtypes of lymphomas are different [3][4][5]. For sinonasal DLBCL, the chemotherapy treatment known as R-CHOP (rituximabcyclophosphamide, doxorubicin, vincristine, and predisone) is most common [3,4]. ...
... Treatment strategies for the two histologic subtypes of lymphomas are different [3][4][5]. For sinonasal DLBCL, the chemotherapy treatment known as R-CHOP (rituximabcyclophosphamide, doxorubicin, vincristine, and predisone) is most common [3,4]. On the other hand, the current standard approach for sinonasal ENKTL is the non-anthracyclinecontaining chemotherapy with or without radiotherapy [5]. ...
PurposeTo evaluate whether imaging features on conventional magnetic resonance imaging (MRI) can differentiate sinonasal extranodal natural killer/T cell lymphomas (ENKTL) from diffuse large B cell lymphoma (DLBCL).Methods
Consecutively, pathology-proven 59 patients with ENKTL and 27 patients with DLBCL in the sinonasal region were included in this study. Imaging features included tumor side, location, margin, pre-contrast T1 and T2 signal intensity and homogeneity, post-contrast enhancement degree and homogeneity, septal enhancement pattern, internal necrosis, mass effect, and adjacent involvements. These imaging features for each ENKTL or DLBCL on total 86 MRI scans were indicated independently by two experienced head and neck radiologists. The MRI-based performance in differential diagnosis of the two types of lymphomas was evaluated by multivariate logistic regression analysis.ResultsAll ENKTLs were located in the nasal cavity, with ill-defined margin, heterogeneous signal intensity, internal necrosis, marked enhancement of solid component on MRI, whereas DLBCLs were more often located in the paranasal sinuses, with MR homogenous intensity, mild enhancement, septal enhancement pattern, and intracranial or orbital involvements (all P < 0.05). Using a combination of location, internal necrosis and septal enhancement pattern of the tumor in multivariate logistic regression analysis, sensitivity, specificity, and accuracy in differential diagnosis of ENKTL and DLBCL were 100%, 79.4%, and 91.9%, respectively, for radiologist 1, and were 98.3%, 81.5%, and 93.0%, respectively, for radiologist 2.ConclusionMRI can effectively differentiate ENKTL from DLBCL in the sinonasal region with a high diagnostic accuracy.
... 15 It affects males in the seventh decade of life. 15,16 Clinically, patients demonstrate nasal obstruction, rhinorrhea, and bloody discharge, as well as B symptoms, such as fever and weight loss. 15 On the basis of these features and the clinical behavior of the lesion, we considered DLBCL as a strong possibility. ...
... PTL is often curable without extensive surgery if it is diagnosed early and treated appropriately [14,15]. Therefore, a suspicion of PTL by the radiologist or clinician is important for the early diagnosis and prompt treatment of this potentially curable thyroid malignancy. ...
Introduction:
The aim of the study is to investigate the usefulness of ultrasound combined with computed tomography (CT) for distinguishing between primary thyroid lymphoma (PTL) and Hashimoto's thyroiditis (HT).
Material and methods:
The investigation was conducted retrospectively in 80 patients from January 2000 to July 2018. All patients underwent pathological test to be classified into two groups: PTL group and HT group. The cut-off value of CT density was determined using receiver-operating characteristic (ROC) curve analysis. The accuracy, sensitivity, specificity, positive predictive value (PPV) and negative predictive value (NPV) of diagnosis for thyroid by CT alone, ultrasound alone, and the combination of CT plus ultrasound were calculated respectively.
Results:
Of the 80 study patients, 27 patients were PTL, 53 patients were HT. Mean CT density had a sensitivity of 90.6 % and a specificity of 88.9 % at a cut off values of 53.5 HU, with area under the curve (AUC) 0.88. Ultrasound combined with CT had the highest specificity, accuracy, PPV compared with CT alone and ultrasound alone (P value <0.05).
Conclusions:
Features such as extremely hypoechogenicty, enhanced posterior echo, cervical lymphadenopathy in ultrasound image and linear high density strand signs, very low density in CT image have high sensitivity and specificity in thyroid lymphoma. Therefore, ultrasound combined with CT may be useful for distinguishing between PTL and HT.
... ENKL predominantly occurs in the extranodal sites, and damages the midline facial structures and other sites including the orbit, salivary glands and paranasal sinuses (1)(2)(3). It is common to observe systemic symptoms (B symptoms: fever, weight loss and night sweats) as well as lymphoma-associated hemophagocytic syndrome in advanced cases (56,57). ...
Extranodal natural killer/T-cell lymphoma, nasal type (ENKL), is a rare malignancy of Non-Hodgkin lymphoma characterized by an aggressive clinical course and poor prognosis. It shows strong association with Epstein-Barr virus infection and occurs more commonly in Asia and Latin America. Various genetic alterations have been identified in ENKL by gene expression profiling and sequencing techniques. The frequent deletion of chromosome 6q21 was reported to lead to the silence of several tumor suppressor genes. Also, there have been novel genetic mutations that were recently uncovered and were found to frequently activate several oncogenic pathways, including the JAK/STAT, NF-κB, and MAPK pathways. Besides, we believe that deregulated single genes and epigenetic dysregulation might be relevant to the mechanism of this disease and thus, may have the potential to shed lights on the development of new therapeutic strategies. The consensus on the standard treatment for ENKL has not yet been currently established. For localized ENKL patients, radiotherapy with concurrent chemotherapy and sequential patterns of chemotherapy and radiotherapy are recommended as first-line therapy. As for advanced or relapsed/refractory ENKL patients, the application of non-anthracycline-containing regimens have significantly improved the clinical outcome, contributing to higher response rate, longer overall survival and progression-free survival. Hematopoietic stem cell transplantation is widely recommended for consolidation after a complete remission or partial remission has been achieved. The anti-programmed death 1 antibody, an immune checkpoint inhibitor, has demonstrated favorable results in treating relapsed or refractory ENKL. Of the current ENKL treatment, researchers are still striving to validate how radiotherapy and chemotherapy should be optimally combined and which of the non-anthracycline-containing regimens is superior. In this review, we summarize the main genetic alterations frequently found in ENKL and their role in providing new insights into the therapeutic targets of this disease, and highlight the recent findings regarding new biologic markers, novel therapeutic strategies applied to this intriguing neoplasm.
