Extrapulmonary Inflammatory Myofibroblastic Tumor Research Articles

Inflammatory Myofibroblastic Tumor of Colon Mimicking Advanced Malignancy: Report of Two Cases with Review of Literature

Inflammatory myofibroblastic tumor (IMT) is mostly found in lungs of children and young adults. However, extrapulmonary IMT can be found in all age groups. A 47-year-old female presented with large anterior abdominal wall mass with fecal fistula from the transverse colon. She underwent wide local excision including tumor mass along with involved parietal wall excision with segmental transverse colectomy. In another case, a 43-year-old male patient presented with acute large intestinal obstruction due to IMT in the sigmoid colon. He underwent emergency subtotal colectomy. The clinical and radiological features of both patients were suggestive of the malignant disease. However, histopathology and immunohistochemistry (IHC) reports were confirmatory of IMT. Their postoperative recovery was uneventful, and they are under regular follow-up for the last 2 years without any evidence of recurrence. On literature review, we found that IMT of the colon is a very rare presentation. Also, patients with IMT of the colon may present differently. It appears like malignancy on clinical and radiological findings, and only histopathology with IHC can confirm its diagnosis. Complete surgical excision is the treatment of choice, and patients need regular follow-up in postoperative period in view of its nature of recurrence.

Extrapulmonary inflammatory myofibroblastic tumor at different sites with histopathology and immunohistochemical analysis: A case series

Background: Inflammatory myofibroblastic tumor (IMT) is a rare entity and has wide anatomical sites of origin. Because of overlapping clinical and radiological features, histopathology study followed by immunohistochemistry (IHC) analysis is necessary for confirmation of the diagnosis. Aim of the Study: This study aimed to present the demographic, histopathological, and IHC analysis of patients diagnosed with extrapulmonary IMT. Materials and Methods: Patients diagnosed with extrapulmonary IMT were analyzed from January 2017 to July 2018. Results: A total of six cases were included in our series, with the mean age of presentation being 37 years. Male-to-female ratio was 1:2. The primary site of origin was distributed as follows: two cases of face and neck (one mandible and one naso-orbital), three cases of abdominal location (one spleen, one periaortic node, and one paraaortic node), and one case of cervix. There was no local invasion or metastasis found. Microscopically, all the cases revealed a common pattern of finding of the presence of intersecting fascicles of spindle cells with intervening collagenous to myxoid matrix along with infiltration of plasma cells and aggregates of lymphocytes. There were no atypical cells or mitosis in all the cases. All six cases were smooth muscle actin and vimentin positive and S100, desmin, and cytokeratin negative on IHC study. All the cases were anaplastic lymphoma kinase (ALK) negative, except one case with cervical primary where it was positive for ALK-1. Conclusion: Extrapulmonary IMT is a rare entity with diagnosis by exclusion on histopathological examination, and IHC should be done for confirmation of the diagnosis as well as ALK expression status.

Inflammatory myofibroblastic tumour: A case report and a clinical update

The Inflammatory myofibroblastic tumor (IMT) is a heterogeneous group of rare lesions consisting predominantly of inflammatory cells and myofibroblastic spindle cells. Head and neck IMTs account for 14 to 18% of extra-pulmonary IMTs [lungs being the most commonly affected regions]. On account of its ambiguous clinical presentation, an IMT needs to be differentiated from other infectious, granulomatous, autoimmune and neoplastic lesions on the basis of histopathologic findings and immunohistochemical analysis. In this article, we report a case of IMT that presented in the anterior mandible that was treated by peripheral resection. Follow-up at 1 year showed satisfactory healing and no signs of recurrence. A special emphasis has been placed on the disputed nosology of this lesion and the latest therapeutic modalities.

Atypical Presentation of Intestinal Intussusception Caused by Insidious Inflammatory Myofibroblastic Tumor of the Bowel Wall

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.

Extrapulmonary inflammatory myofibroblastic tumor with synchronous involvement of the liver and appendix

Inflammatory myofibroblastic tumor (IMT) of the liver is a rare disease, often confused with hepatic neoplasm. A 60-year-old man underwent liver resection for suspected hepatocellular carcinoma with concurrent appendicectomy for incidentally detected diseased appendix. Histopathology revealed IMT involving both the liver and appendix. We present the diagnostic challenge and approach in a patient with IMT of the liver with the synchronous incidental involvement of appendix treated successfully by liver resection and appendicectomy.

Inflammatory Myofibroblastic Tumor of the Lung and the Maxillary Region: A Benign Lesion with Aggressive Behavior

Inflammatory myofibroblastic tumor (IMT) is a rare mass-forming lesion characterized by fibroblastic or myofibroblastic spindle cell proliferation with varying degrees of inflammatory cell infiltration. Although it has been reported in virtually every organ in the body, the lung is the most common site of involvement. Extrapulmonary IMTs, although rare, have been reported and are characterized by different, more aggressive behavior. We report an extremely rare case of maxillary metastases of pulmonary IMT. Lung IMT was initially misdiagnosed, and oral lesion mimicked clinically and radiologically a radicular cyst. On histologic examination, cells exhibited diffuse and intense immunoreactivity for α-smooth muscle actin and vimentin whereas both pulmonary and oral IMTs presented absence of cellular atypia and lack of expressivity of oncogenic determinants. Distant metastases of lung IMT are extremely unusual, and this is the first report to our knowledge with this particular clinical course. Despite the possibility that the present case could also represent a metachronous multifocal IMT, with pulmonary and extrapulmonary lesions, similar histopathological and immunohistochemical patterns in lung and maxillary region suggest a metastatic course.

Inflammatory Myofibroblastic Tumor of the Esophagus

Inflammatory myofibroblastic tumors are regarded as intermediate-grade tumors with a potential for recurrence. Although these lesions have been found in nearly every anatomic location, there are few documented cases of esophageal localization. The rare case reported here concerns a 55-year-old woman with an extremely large inflammatory myofibroblastic tumor of the esophagus; the tumor was 20 cm in length and 6 cm in diameter. Wide surgical excision was performed. Histopathologic and immunohistochemical analyses confirmed the diagnosis of an inflammatory myofibroblastic tumor. There was no recurrence or tumor metastasis 6 months after the operation.

Inflammatory Myofibroblastic Tumor Presenting With Esophageal Obstruction and an Inflammatory Syndrome

Inflammatory myofibroblastic tumor is a rare neoplasm of intermediate malignant potential. Although inflammatory myofibroblastic tumor occurs at multiple anatomic locations, an esophageal lesion is extremely rare. We describe a 43-year-old man who presented with severe dysphagia and an inflammatory syndrome, secondary to esophageal inflammatory myofibroblastic tumor. The patient was treated successfully with esophagectomy and remains disease free at 1 year. This case illustrates the complexities involved in managing a large esophageal myofibroblastic tumor and highlights that esophagectomy, rather than enucleation, should be the treatment of choice.

Inflammatory Myofibroblastic Tumor of the Abdominal Wall Simulating Rhabdomyosarcoma: Report of a Case

We report the case of a 6-year-old boy with a progressively enlarging mass in the anterior abdominal wall. Computed tomography showed an intensely enhancing heterogeneous lesion arising from the anterior abdominal wall muscles with intraperitoneal extension. Based on the location of the tumor and the radiological findings we made a provisional diagnosis of rhabdomyosarcoma. However, the findings of fine-needle aspiration and Trucut biopsy were inconclusive for malignancy. We performed complete excision of the mass, including the involved left costal segment. Microscopically, the tumor was composed mainly of spindle-shaped cells without malignant features, and immunohistochemical analysis revealed positive staining for actin. These findings confirmed a diagnosis of an inflammatory myofibroblastic tumor (IMT). To our knowledge, this is the first case report of an extrapulmonary IMT arising from the anterior abdominal wall.