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Advanced Technical IVT

desmoid tumor

Pronunciation: DEZ-moyd

A rare, locally aggressive fibrous tumor that can occur sporadically or in association with familial adenomatous polyposis syndrome.

Full Definition

Desmoid tumors, also known as aggressive fibromatosis, are rare, locally invasive fibrous neoplasms that arise from musculoaponeurotic structures. While histologically benign, these tumors exhibit aggressive local growth and have a high propensity for local recurrence after incomplete excision. In the context of gastrointestinal oncology, desmoid tumors are particularly significant because they occur in 10-25% of patients with familial adenomatous polyposis (FAP), often developing after colectomy. These FAP-associated desmoids can arise in the mesentery or abdominal wall and may become life-threatening due to bowel obstruction or compression of vital structures. Treatment options include observation, medical therapy with NSAIDs or hormonal agents, radiation therapy, and surgical resection when feasible.

Usage

Usage note: Distinguish from malignant sarcomas; note association with FAP syndrome.

In Context

  • "The patient with FAP developed a large intra-abdominal desmoid tumor two years after prophylactic colectomy." — Genetic counseling note
  • "Medical management with celecoxib was initiated for the desmoid tumor given its unresectable location near the superior mesenteric vessels." — Oncology treatment plan

Also known as

aggressive fibromatosis

Don't confuse with

fibrosarcoma GIST

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