Giant aggressive mesenteric fibromatosis- a case report

Abstract

Fibromatosis are rare, accounting for 0.03% of all tumours. Mesenteric fibromatosis is a very rare (8% of all desmoid neoplasm). Aggressive fibromatosis of mesentery is a rare surgical problem affecting 2-4 per million people. Females are more commonly affected than males (Estrogen acts as a growth factor). It is locally invasive and tends to recur but never metastasize. Here, we are discussing about 24-year-old male presented with progressive abdomen distension associated with pain since one month. Abdominal examination showed a firm non-tender intra-abdominal mass, measuring around 15x14 cm size, with intrinsic mobility, which was perpendicular to mesenteric line, all borders were well-made out. CECT abdomen showed features suggestive of GIST . Elective Laparotomy was done and a giant mass arising from mesentery without any infiltration to the surrounding structures was noted. The entire mass was excised and mesentery repaired. Histopathology showed uniform band of spindle shaped cells arranged in fascicles admixed with blood vessels in a collagenous stroma. Immunohistochemistry showed Beta Catenin +ve, CD 117-ve, CD 34 -ve and SMA-ve, which is confirmative of Fibromatosis. Postoperative period was uneventful.

Keywords: Beta catenin; Desmoid tumour; Estrogen; Gist.

[Table/Fig-1a,b]:

A Large well-defined lesion noted in abdominal cavity from L2-L5 level just posterior to anterior abdominal wall. Lesion measures 16.4×15.2 ×10.6 cms. Post contrast, Enhancement of the lesion was noted with central non-enhancing necrotic areas. No evidence of calcification's within the lesion. Differential Diagnosis GIST / Mesenteric Tumour

[Table/Fig-2a]:

Giant tumour arising from Mesentery of small intestine

[Table/Fig-2b]:

Mesentery of small bowel after complete excision of tumour

[Table/Fig-2c]:

Gross Specimen of Mesenteric Fibromatosis

[Table/Fig-3a,b]:

(Histopathology) Uniform band of spindle shaped cells arranged in fascicles admixed with blood vessels in a collagenous stroma. Cells are infiltrating skeletal muscle without necrosis and mitosis

[Table/Fig-4]:

Immunohistochemistry positive for Beta Catenin