A Large Sporadic Intra-abdominal Desmoid-Type Fibromatosis in a Young Male: A Case Report

Abstract

Desmoid type fibromatosis (DTF) is a rare benign tumor of connective tissue origin. While these tumors are typically not malignant, they can exhibit aggressive growth patterns causing mass effect on surrounding organs. These tumors typically present in the extremities and abdominal wall, rarely occurring in the mesentery, and abdominal organs. Due to the rarity of this tumor and the variable size and origin, it is difficult to provide exact prognosis, recurrence, and treatment efficacy regarding desmoid tumors arising from the ileocolic mesentery. We present a case of a young male with a sporadic 31 × 25 × 12 cm DTF arising from the ileocolic mesentery that was complicated by mass effect on bowel and intra-abdominal organs requiring surgical intervention. On presentation, the patient exhibited weight gain and abdominal pressure. Abdominal distension without tenderness on palpation was noted on physical examination. The tumor biopsy confirmed the diagnosis of DTF. No evidence of familial adenomatous polyposis or Gardner syndrome was identified. The tumor was surgically excised and intimately associated with the bowel requiring ileocolonic resection with primary anastomosis. At 3-months follow up, surveillance MRI showed no residual or recurrent lesion. A multidisciplinary approach to this patient's diagnosis and treatment allowed for an accurate diagnosis, efficient treatment, and follow up plan.

Giant abdominopelvic desmoid tumour herniated trough perineum: a case report

“…In a study by Xiao et al ., only 1 of 16 patients had a mass whose diameter was >20 cm. Although large desmoids have been previously reported [ 4 ], none had abdominal pelvic and perineal features. The diagnostic workup for large desmoid tumours is challenging.…”

“…Regardless of the aetiology, local growth and recurrence are therapeutic problems in desmoid tumours. The treatment options include surgery, radiotherapy, chemotherapy, hormonal treatments and more recent targeted therapies [ 3 , 4 ]. These treatments remain poorly documented because of the disease rarity and inconclusive results.…”

Cytoreductive surgery for giant locally advanced intra-abdominal tumors in Uganda

“…Giant intra-abdominal tumors exert a lot of pressure symptoms and this can be uncomfortable just like in our patient’s case [ 6 ]. These tumors present with complications, such as in Patient 3, who had lower limb swelling with deep venous thrombosis from the tumor’s pressure on the left common iliac vein.…”

An accidental finding of a retroperitoneal desmoid tumor: case report and review of the literature

“…Retroperitoneal desmoid-type fibromatosis (DTF) is a rare benign mesenchymal neoplasm that develops as a result of fibroblastic proliferation within the musculoaponeurotic stroma 1 , 2 . This tumor may infiltrate the surrounding tissue, but hardily metastasize distantly 3 .…”

“…DTF is found to occur in multiple locations, but they most commonly arise from the abdominal wall, mesentery, and the extremities followed by the abdominal cavity and retroperitoneum 4 , 5 . It can be hereditary or sporadic, with the latter being associated with familial adenomatous polyposis (FAP) or Gardner’s syndrome 2 . Incidence has been reported to occur in 7.5–16% of FAP patients or those with Gardner’s syndrome.…”