Familial desmoids in association with adrenal and ovarian masses and leiomyomas - Report of three gases

PURPOSE: Desmoid tumors are rare occurrences. However, they are more common ly seen in patients with familial adenomatous polyposis. The purpose of thi s article is to review three cases of abdominal desmoids in association wit h adrenal, ovarian masses, leiomyoma, and neurologic involvement in three s isters with familiar adenomatous polyposis. METHOD: ri case study was done of these sisters, their siblings, and their parents. RESULTS: AU three sist ers had desmoid tumors and no evidence of familial adenomatous polyposis. A il three sisters had ovarian pathology, two had adrenal masses, one had a f ibroadenoma of the breast, one had a leiomyoma of the stomach, and two had neurologic involvement. Additionally, male siblings, parents, and grandpare nts had no evidence of desmoids or familial adenomatous polyposis. CONCLUSI ON: This constellation of symptoms in the affected sisters may reflect a se parate gene defect predisposing to desmoid tumors or a varying expression o f the adenomatous polyposis coli gene.