Desmoid tumors are rare neoplasms. They occur mostly in females in their reproductive age and may present with pelvic pain and intestinal obstruction. These connective tissue neoplasms are because of uncontrolled proliferation of differentiated myofibroblasts. The cells may synthesize vast amounts of collagen fibers in response to various stimuli. We describe a case of a pelvic desmoid tumor simulating a uterine leiomyoma recurrence. We review the literature on the epidemiology and the treatment options for desmoid tumors and suggest a strong index of suspicion when a new pelvic mass arises in an adult with previous pelvic surgery. We advise a planned multidisciplinary treatment approach at the first hint of the diagnosis of desmoid tumor.
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