A huge low-grade fibromyxoid sarcoma of small bowel mesentery simulating hyper immune splenomegaly syndrome: A case report and review of literature

OI Alatise, OA Oke, OO Olaofe, GO Omoniyi-Esan, ARK Adesunkanmi

Abstract

Introduction: Low-grade fibromyxoid sarcoma (LGFMS) is a rare non epithelial tumour. It usually arises from the smooth muscles of the extremities. It is, however, occasionally reported to arise from other regions of the body.

Case report: We report the case of a 32 year old man who complained of a progressive abdominal swelling of 4 months duration. There was associated abdominal discomfort and weight loss. Abdominal examination revealed a non-tender intra abdominal mass filling the abdomen completely. Abdominal ultrasound suggested a massive splenomegaly. Abdomina Computerized Tomography (CT) scan was not done due to financial constraints. At laparotomy, a large, pearl-coloured mass was found within the mesentery of the proximal jejunum, with dilated, tortuous vessels. It was resected along with the overlying 60cm of jejunum. It weighed 7.5kg. Histology and immunohistochemistry confirmed the diagnosis of lowgrade fibromyxoid sarcoma. Post-operative period was uneventful and there were no features of recurrent after 2 year of follow up.

Conclusion: LGFMS may cause a diagnostic dilemma, especially in a third world setting where preoperative diagnosis is hampered by lack of facilities and poverty. A high index of suspicion is needed for preoperative  diagnosis, which is necessary for proper planning of the operation.

Key word: Low grade fibromyxoid sarcoma, Nigeria, Hyper immune splenomegaly syndrome