Abstract
Desmoid tumors (DTs) are rare soft tissue neoplasms, especially when originating from the small bowel. An association with familial adenomatous polyposis (FAP) of the colon has been well documented. Within the FAP population, there is a strong correlation between prophylactic procto-colectomy and subsequent development of DTs. Very few reports describe cases of FAP initially presenting with desmoid-related complications. Therefore, we underwent a review of the literature in order to delineate the characteristics of desmoid tumors occurring as an initial presentation of FAP.
Highlights
BackgroundDesmoid tumors or fibromatosis are rare soft tissue neoplasms, especially when originating from the small bowel
Secondary Desmoid tumors (DTs) are commonly associated with hereditary polyposis syndromes with 30% of familial adenomatous polyposis (FAP) patients developing intraabdominal desmoids disease, including tumors, nodules, and sheet-like lesions
Small bowel obstruction (SBO) is a common complication of desmoid tumors which is reported in 27% to 58% of patients with FAP-related desmoid tumors in previous studies
Summary
Desmoid tumors or fibromatosis are rare soft tissue neoplasms, especially when originating from the small bowel They commonly affect the mesenchymal tissues intra-abdominally, within the abdominal wall or in the limb girdle. Secondary DTs are commonly associated with hereditary polyposis syndromes with 30% of FAP patients developing intraabdominal desmoids disease, including tumors, nodules, and sheet-like lesions. They can occur secondary to trauma or hormonal stimulation [1,2]. It is uncommon for FAP to initially present with SBO secondary to DT In this setting, SBO can be caused either by direct mass effect due to tumor growth, or infiltration of the mesentery by tumor cells causing wrinkling of the bowel margin and sclerosis of the mesenteric vessels which leads to ischemic stricture formation. In cases of retroperitoneal involvement, DTs could result in major blood vessels compression and ureteral obstruction [67]
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