Inflammatory myofibroblastic tumour is a clinico-pathological condition involving inflammatory cells embedded within myofibroblastic proliferation. This condition has been commonly cited in mediastinum with rare incidence in the gastro-intestinal tract. This… Click to show full abstract
Inflammatory myofibroblastic tumour is a clinico-pathological condition involving inflammatory cells embedded within myofibroblastic proliferation. This condition has been commonly cited in mediastinum with rare incidence in the gastro-intestinal tract. This report intends to highlight the therapeutic dilemma in management, as clinical and radiological features may not be sufficient to distinguish these rare tumours from malignancy. A 30-year-old male presented with 2 weeks of continuous and progressive lower abdominal pain that shifted to right lower quadrant (RLQ) pain since 2 days. Clinical examination was consistent with tenderness and guarding. Computed tomography scan was consistent with appendicolith and peri-appendiceal inflammation (Fig. 1). Laparoscopy revealed a well-delineated mass replacing appendix. Mesenteric adenopathy was not evident and rest of the abdomen was unremarkable. Laparoscopic en bloc resection of mass along with a wedge of cecum was performed. In vitro cut section revealed the outline of appendix embedded inside a homogenous yellow mass with associated fecolith (Fig. 2). Post-operative stay was uneventful. Patient continues to be asymptomatic and follow-up computed tomography scan after 34 months did not show evidence of recurrence (Fig. 3). The World Health Organization has classified inflammatory myofibroblastic tumours as intermediate biologic potential tumours due to tendency for local recurrence and minimal risk of distant metastasis. Accurate incidence and prevalence is difficult to obtain due to interchangeable use of ‘inflammatory pseudo tumor’ and ‘inflammatory myofibrobalstic tumor’ as well as due to evolving nomenclature. These tumours have been reported in virtually all the organs. The clinical symptoms may be organ specific or compressive and constitutional syndrome including fever, malaise and weight loss is seen in 15–30% of patients. Hematologic abnormalities may include microcytic anaemia, thrombocytosis and elevated erythrocyte sedimentation rate. Biochemically, patient may have polycolonal hypergammaglobulinemia. Radiological findings are polymorphic with variability in density, contrast enhancement and homogeneity. The definitive diagnosis is made by surgical excision and histologic/immunohistochemistry analysis. Histologically, they are considered as pseudosarcomatous proliferation of the spindle shaped myofibroblasts intermingled with inflammatory cells. As adjuvant therapy has minimal effect, importance is placed on recognition and complete excision. Lesions simulating malignancy with microscopic/immunohistochemical features of inflammatory myofibroblastic tumour is a rare
               
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