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Introduction/Objective Inflammatory myofibroblastic tumor (IMT) is an uncommon spindle cell lesion that can involve various organs and occurs in multiple body sites. While older terminology (i.e. inflammatory pseudotumor) suggested otherwise, recent molecular studies point toward a neoplastic pathogenesis for IMTs. Herein, we report a case of an abdominal IMT and discuss the morphologic and immunohistochemical pitfalls pertaining to this entity. Methods A 75-year-old woman presented with complaints of generalized abdominal pain and distention. An abdominal CT scan showed multiple peritoneal masses, the largest of which measured 23 cm. Biopsy revealed compact fascicles of bland spindle cells exhibiting diffuse actin and caldesmon immunoreactivity, consistent with a spindle cell tumor with smooth muscle differentiation. Mitotic activity was low-to-unapparent. Surgical excision was performed. The cut surface of the tumor was tan-white with hemorrhagic foci. Histopathologic examination of the tumor showed elongated spindle cells set in a loose myxoid stroma rich in blood vessels and a mixed inflammatory infiltrate. Deeper sections of the tumor were more cellular, showing a similar morphology to that seen in the original biopsy, which was virtually devoid of inflammatory cells. Immunohistochemistry showed diffuse staining for desmin, caldesmon, smooth muscle actin, and ALK. FISH analysis showed ALK gene rearrangement in 52% of tumor cells, confirming the diagnosis of IMT. Results Studies in the literature show that IMTs express smooth muscle markers such as SMA (90%) and desmin (50%). However, immunoreactivity for caldesmon is rarely reported. ALK immunoreactivity is seen in about 35–60% of cases, and when gene rearrangement involving ALK is detected the diagnosis can be confirmed. Conclusion Historically, several terms have been used to describe IMTs, including inflammatory pseudotumor and inflammatory pseudosarcomatous fibromyxoid tumor. While the nomenclature consistently implies an inflammatory infiltrate, this tumor could have various morphological patterns with some areas showing very little to absent inflammation, as demonstrated in our case. Hence, making an accurate diagnosis could be challenging on a limited biopsy. Therefore, ALK testing should be included as part of the diagnostic workup of spindle cell neoplasms with smooth muscle differentiation.

Introduction: Paraneoplastic pemphigus is an autoimmune disease associated with an underlying tumor. Several cases have been reported as unusual pemphigus vulgaris, erythema multiforme, or paraneoplastic bullous disease,1 but the incidence of the condition is unknown.2 Response to treatment is generally poor, with significant morbidity and mortality. This condition is clinically characterized by severe mucositis and polymorphic blistering skin eruptions, and histologically by acantholysis, keratinocyte necrosis, and interface dermatitis.3 Immunoprecipitation and immunoblot testing detect autoantibodies against desmosomal polypeptides.4 We describe a case of paraneoplastic pemphigus associated with an abdominal inflammatory myofibroblastic tumor in a 7-year-old girl.

An inflammatory myofibroblastic tumor, also known as inflammatory pseudotumor, is a rare neoplasm characterized by myofibroblastic spindle and inflammatory cells that cause masses in many sites of body. It is often benign, but in some cases neoplastic transformation has been reported as a result of aggressive growing. In our case, an inflammatory myofibroblastic tumor was reported by biopsy of a 25 × 15 cm abdominal mass. How to cite this article Tastekin F, Ersoy M, Temel T, Ozgenel SM, Canaz F, Özakyol A. Abdominal Inflammatory Myofibroblastic Tumor: A Rare Case. Euroasian J Hepato-Gastroenterol 2016;6(2):183-185.

Inflammatory myofibroblastic tumors (IMT) are lesions that mostly affect young adults and children. The tumor is made up of myofibroblasts and a mixed inflammatory infiltrate and rarely undergoes malignant transformation. We present a case of a 13-year-old boy with a pelvic mass diagnosed as IMT which underwent malignant transformation and metastasised to the liver. We report the ultrasound, computed tomography (CT) and magnetic resonance (MR) findings of this rare disorder.