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IMFT displaying a dense, spindle cell myofibroblastic infiltrate with admixed plasma cells, lymphocytes and eosinophils (haematoxylin and eosin (240X)).
Source publication
Inflammatory myofibroblastic tumours (IMFTs) are rare tumours characterised by nosologic, histogenetic and aetiopathogenetic controversy and variable clinicopathological features. We report our experience with intestinal-IMFTs (I-IMFTs) that have been reported mainly as single case reports to date.
Five patients with I-IMFTs, identified between 200...
Citations
... Inflammatory myofibroblastic tumour (IMFT) is an uncommon tumour predominantly affecting the younger age group with predilection inside the thoracic cavity. Its intra-abdominal involvement is sporadic [1]. ...
IMFT (inflammatory myofibroblastic tumour) is an uncommon tumour predominantly affecting the lungs and mediastinum. Most of the published literature supports that it affects children and young individuals. IMFT involving the gastrointestinal tract is rare. We report a case of multifocal IMFT affecting the GI tract which was managed with gross total excision followed by chemotherapy. Surgical resection remains the treatment of choice. The role of chemotherapy and radiation therapy remains limited. The aetiology of these tumours remains unclear and is mostly ALK-positive that could be targeted. Local recurrences are common and hence require close follow-up. The risk of recurrences and metastasis is increased in cases with TP53 positivity, aneuploidy and recurrent lesions.
... Corticosteroids can be added to reduce the inflammatory reaction. Extrapulmonary location, especially in the abdomen, locally invasive lesions, and more than 8 cm tumor size, often has a high propensity for recurrence [8]. The recurrence rate is meager and regular follow-up is necessary for the long term to avoid it. ...
Inflammatory myofibroblastic tumors (IMFTs) are rare solid mesenchymal tumors frequently noted in children and young adults. It is characterized by variable clinicopathological and etiopathogenetic features. They are commonly reported in the lungs and occurrence in the colon is extremely rare. Here, we report a case of IMFT in the sigmoid colon confirmed histopathologically after surgical resection.
A 40-year-old lady presented with abdominal pain, vomiting, and constipation for four days. On abdominal examination, there was tenderness in the left iliac fossa region with localized guarding. Contrast-enhanced computed tomography (CECT) showed a sigmoid colonic mass lesion with few enlarged perilesional lymph nodes. Colonoscopy demonstrated circumferential ulceration with irregular margin associated with luminal narrowing noted 55 cm from the anal verge and scope could not negotiate beyond, biopsies were taken. Later, the biopsy came as descriptive in nature. Hence, we proceeded for surgery and intra-operatively we have found there was circumferential thickening in the sigmoid colon for about size 8 cm of which was abutting the left lateral parietal wall. We have done sigmoid colon resection with adequate margins and postoperatively patient did well. Finally, the histopathology report suggested an IMFT sigmoid colon.
... It has been also reported, in 30% of patients with IMT, that the presence of the tumor was associated with recurrent infections caused by mycoplasma, nocardia, actinomycetes, EBV or human herpes virus species [6,12]. In addition, trauma, abdominal surgery, and genetic factors have been associated with the presence of In ammatory myo broblastic tumor [13]. No previous history of trauma or abdominal surgery was reported in the presented case. ...
Inflammatory myofibroblastic tumor (IMT) is an uncommon neoplasm that has been described in various locations throughout the body, but is rarely observed in systemic lymph nodes. We present a case of a 63 years old woman with left inguinal lymphadenopathy accompanied by low-grade fever. Fluorine-18-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG PET/CT) revealed abnormal higher18F-FDG uptake on the neck, axillar, pulmonary hilar, mediastinal, mesenteric, retroperitoneal, pelvic and inguinal lymph nodes. These findings led to an initial misdiagnosis of lymphoma. Final histological diagnosis revealed an IMT. The patient was treated with oral steroids. Ultrasound assessments showed a complete resolution of systemic enlarged lymph nodes at the end of 1 month of therapy. There has been no evidence of recurrence through 12 months of post-treatment monitoring. This case suggests that IMT should be considered as a possible differential diagnosis in apparent cases of lymphoma. Further, it indicates that steroid therapy may serve as an effective treatment for IMTs that systemically affect lymph nodes.
... However, the tumor in the gastrointestinal tract accompanied by intussusception appears to be rare. [3][4][5][6][7][8][9][10][11][12][13][14][15] We documented this rare case of a 12-year-old girl presenting with abdominal pain. The clinical and radiologic impression was bowel intussusception and bowel obstruction. ...
... 16 However, intestinal intussusception caused by inflammatory myofibroblastic tumor (IMT) was rare. [3][4][5][6][7][8][9][10][11][12][13][14][15] Intestinal IMT (I-IMT) can be served as a lead point of intussusceptum, cause bowel disorders, and result in intestinal intussusception. ...
... IMT was first described in the lung, and multiple extrapulmonary manifestations have been reported. [2][3][4][5][6][7][8][9][10][11][12][13][14][15] Coffin et al showed that the most common sites of extrapulmonary IMT were the mesentery and omentum in 36 of 84 cases (43%); only 1 case(1.2%) exhibited an IMT of ileal origin. 2 In the presented case, a tumor arose from the bowel wall of the ileum and appeared to be rare. ...
Introduction
Inflammatory myofibroblastic tumor (IMT) is a rare but real tumor, which is histologically characterized by myofibroblastic spindle cells proliferation with inflammatory infiltrate. The lung is the most common affected organ, and extrapulmonary IMTs are less common. However, IMT seldom presents in the gastrointestinal tract, and intussusception is a rare complication of this tumor.
Case presentation
We documented this rare case of a 12-year-old Chinese girl presenting with abdominal pain. The clinical and radiologic impression was bowel intussusception and bowel obstruction. No sign of abdominal mass was found before surgery, neither physical examination nor radiologic images. Operative findings revealed intestinal intussusception secondary to a little mass. Histopathlogic evaluation of this mass revealed IMT.
Conclusion
In conclusion, IMT may present with bowel intussusception. However, at the intestinal location, the tumor may be found as an abdominal mass or may be insidious; hence, detailed history, physical examination, and imaging studies are necessary for early recognition and diagnosis.
... IMT, formerly known as inflammatory pseudotumor or plasma cell granuloma, is composed of a variable mixture of collagen, inflammatory cells, and usually cytologically bland spindle cells showing myofibroblastic differentiation. It has been recently reclassified as a malignant tumor [15]. It is described as usually localized, with uncommon invasion of the thoracic wall, mediastinum or pleura, and infrequent recurrence or distant dissemination [15][16][17]. ...
... It has been recently reclassified as a malignant tumor [15]. It is described as usually localized, with uncommon invasion of the thoracic wall, mediastinum or pleura, and infrequent recurrence or distant dissemination [15][16][17]. However, in the present series, two patients (15 %) presented with distant lesions [18]. ...
Purpose
Primary lung tumors are rare during childhood and encompass a wide variety of histological types. Each has a different biology and a different therapeutic approach. The aim of this article is to review the experience of a pediatric referral center with this kind of tumors during the last 24 years.
Methods
A retrospective chart review was performed for patients with diagnosis of primary lung tumor between the years 1990–2014. The variables analyzed were age, sex, course of the disease, symptoms, localization, surgery, histology and outcome.
Results
Between 1990 and 2014, 38 patients with primary lung tumors were treated at our institution. Age at presentation was 6.6 ± 5.2 years (r 0.91–16.58) and the female:male relationship was 1.37. Inflammatory myofibroblastic lung tumor (n = 13), carcinoid tumor (n = 6) and pleuropulmonary blastoma (n = 6) were the most frequent histological types. Persistent radiographic abnormality was the most frequent presenting sign (34 %). Global mortality was 15.8 % varying according to histology.
Conclusion
Although the diagnosis of primary lung tumor is rare, the persistence of a radiographic abnormality in spite of adequate treatment for inflammatory processes forces us to evaluate further. The age of the patient is an important factor in the decision of the diagnostic work-up.
... Inflammatory myofibroblastic tumors (IMTs), originally termed as inflammatory pseudotumors, are rare neoplastic lesions with a tendency for locally aggressive behavior and recurrence (1,2). Due to the rarity of IMTs, only a few cases have been reported in the literature to date (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17), and the incidence rates remain unclear. IMTs are most prevalent in the pulmonary system of children and young adults, however, they may also develop in older patients and in other organs (18). ...
... Typically, the most frequent early symptoms presented in patients are a palpable mass, weight loss and/or fever, and abdominal pain. Laboratory abnormalities are exhibited in a small number of patients (15,16). The current case presented clinically with long-lasting abdominal pain that had recently worsened to cramping. ...
... It is mainly agreed that surgery is the predominant treatment modality for IMTs. However, a few studies have reported certain benefits in the treatment of invasive or incompletely resected IMT using immunosuppressive therapy with corticosteroids and non-steroidal inflammatory agents (10,15), radiation therapy (16) and chemotherapy (2,5,16). In the aggressive forms of IMT, exhibiting a high local recurrence rate and a potential for metastasis, it is recommended that close follow-up consisting of physical examination, radiographic imaging and the evaluation of serial erythrocyte sedimentation rates is undertaken (15). ...
Inflammatory myofibroblastic tumors (IMTs) are rare neoplastic lesions with a tendency for locally aggressive behavior and recurrence. IMTs most frequently occur in the soft tissues of children and young adults, with the lungs being the most commonly affected site; however, it has been recognized that any anatomical location may be involved. IMT in the jejunum is extremely rare, with only one case previously reported in the literature. The current study describes the case of a 42-year-old woman presenting with intermittent abdominal pain and small bowel intussusception that was identified during a laparotomy. Surgical resection of the jejunum, revealing 3 exophytic tumors, provided specimens for analysis. Following histological examination, a diagnosis of IMT was made. A review of the literature regarding this rare disease is also presented to emphasize the risk of local recurrence and the importance of adequate long-term follow-up.
... The disease appears to results from diverse etiologies, including Epstein-Barr virus, human herpes virus 8, elevated levels of cytokines (i.e., interleukin-6), 9 reperative, postoperative, and mycobacterial infections, 2 and post-traumatic injury, to name a few. 10 IMT occurred in our case even without a history of abdominal trauma or surgery. Using immunocytochemical techniques, Freeman et al. 11 reported anaplastic lymphoma kinase (ALK) 1 in the urinary bladder of IMT patients (eight/nine cases). ...
... Clinically, intra-abdominal or retroperitoneal tumors present as palpable masses, with symptoms of abdominal pain and weight loss that can be obstructive, 13 and diarrhea in children. 10 Moreover, lung tumors present as dyspnea, pain, fever, and cough. 14 Ntloko et al. 10 reported that the most common symptoms/signs of IMT are abdominal pain and change in intestinal habits; however, our case presented with nonspecific symptoms such as acute abdominal pain and no vomiting or intestinal obstruction. ...
INTRODUCTION
Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm of various anatomical sites, which is histopathologically characterized by spindle-shaped cells with myofibroblastic proliferation and inflammatory infiltration.
PRESENTATION OF CASE
In this case report, a 37-year-old man presented with nonspecific systemic symptoms, including abdominal pain and weakness, which was diagnosed by multislice computed tomography and ultrasonography. An 8 cm × 5 cm × 5 cm nodular gray-white firm noninfiltrative mass, which was well localized in the mesentery tissues of small bowel, was observed and the patient underwent surgical resection.
DISCUSSION
A review of the literature on IMT of the small-bowel mesentery yielded a small number of previously described cases. This tumor most frequently involves the lungs and arises most commonly in extrapulmonary locations such as the mesentery and omentum. The etiopathogenesis and the clinical course of the disease are unclear. The histological and clinical differential diagnosis of IMT also includes reactive processes and mesenchymal tumors of the gastrointestinal tract. Follow-up after surgical removal documented local recurrence and metastasis.
CONCLUSION
The preferred primary treatment is complete surgical excision, and patients require close clinicoradiological follow-up. In general, cases treated with complete surgical resection have a good prognosis.
... The majority of extrapulmonary IMTs, including colonic IMTs, are successfully curable by surgical resection without the development of recurrence (3,6). The prognosis of an IMT of the digestive tract is usually good (3), however, certain cases of IMT that originate from other organs may show recurrence or even metastasis following surgery (7)(8)(9)(10)(11). ...
Inflammatory myofibroblastic tumor is a rare type of lesion that mimics malignancy and has various clinical manifestations. The current study presents a 36-year-old female with a colonic mass, which closely resembled a stromal tumor during imaging. The patient experienced intermittent fever and slight abdominal pain for one month. The fever remained at ≤38.5°C until the day of surgery. The patient underwent a right hemicolectomy and the preoperative fever disappeared and did not recur until the patient was discharged.
... In our study, adenocarcinoma had the highest incidence among the malignant neoplasms, while lipoma was the most common benign tumor. Some extremely rare tumors reported only in single cases [11,12] , including duodenal gangliocytic paraganglioma, jejunal mesangial fibrosarcoma, jejunal inflammatory myofibroblastic tumor and jejunal serosa fibromatosis, were also detected in our group. There are differences in reports regarding the incidence of small bowel tumors. ...
To analyze the clinical characteristics of small bowel tumors detected by double-balloon enteroscopy (DBE) and to evaluate the diagnostic value of DBE in tumors.
Four hundred and forty consecutive DBE examinations were performed in 400 patients (250 males and 150 females, mean age 46.9 ± 16.3 years, range 14-86 years) between January 2007 and April 2012. Of these, 252 patients underwent the antegrade approach, and 188 patients underwent the retrograde approach. All the patients enrolled in our study were suspected of having small bowel diseases with a negative etiological diagnosis following other routine examinations, such as upper and lower gastrointestinal endoscopy and radiography tests. Data on tumors, such as clinical information, endoscopic findings and operation results, were retrospectively collected.
Small bowel tumors were diagnosed in 78 patients, of whom 67 were diagnosed using DBE, resulting in a diagnostic yield of 16.8% (67/400); the other 11 patients had negative DBE findings and were diagnosed through surgery or capsule endoscopy. Adenocarcinoma (29.5%, 23/78), gastrointestinal stromal tumor (24.4%, 19/78) and lymphoma (15.4%, 12/78) were the most common tumors. Among the 78 tumors, 60.3% (47/78) were located in the jejunum, and the overall number of malignant tumors was 74.4% (58/78). DBE examinations were frequently performed in patients with obscure gastrointestinal bleeding (47.4%) and abdominal pain (24.4%). The positive detection rate for DBE in the 78 patients with small bowel tumors was 85.9% (67/78), which was higher than that of a computed tomography scan (72.9%, 51/70). Based on the operation results, the accuracy rates of DBE for locating small bowel neoplasms, such as adenocarcinoma, gastrointestinal stromal tumor and lymphoma, were 94.4%, 100% and 100%, respectively. The positive biopsy rates for adenocarcinoma and lymphoma were 71.4% and 60%, respectively.
DBE is a useful diagnostic tool with high clinical practice value and should be considered the gold standard for the investigation of small bowel tumors.
... Among extrapulmonary IMTs, 43% of the sites arise from mesentery and omentum whereas the small bowel accounts for only 1.2% of the affected sites (1). Moreover, IMTs arising from the small bowel causing intussusception are rare (2,3). Intussusception is primarily a disease among infants and children, and about 5% of the cases occur in adults. ...
Inflammatory myofibroblastic tumor (IMT) of the ileum rarely develops and moreover, intussusception of the tumor is even rarer. We report a case of IMT of the ileum with ileoileal intussusception in which we obtained multi-detector computed tomography images that revealed dilatation of the proximal ileum as well as luminal mass lesion in the distal ileum with intussusceptum.
