• Soft-tissue neoplasms that originate from aponeurotic tissues
• Referred to as "aggressive fibromatosis" and behave as low-grade malignant lesions
• Locally aggressive but virtually never metastasize
• Present as enlarging, often painless, soft-tissue mass
• Most often occur in abdominal wall scars, especially in a cesarean section incision
• Strongly associated with familial polyposis syndromes
• Intra-abdominal desmoids occur most commonly in the setting of familial adenomatous polyposis (FAP)
• Strong female predominance, presentation 1-2 years after parturition, and reports of spontaneous degeneration after menopause all suggest a strong hormonal component to desmoid development
• Desmoids occur with increased frequency in patients with FAP, classically in the mesentery following total proctocolectomy
• Sporadic desmoid formation: enlarging, often painless, soft-tissue mass occurring in or near the vicinity of an incision
• Familial polyposis syndrome following total proctocolectomy: abdominal mass formation or small bowel obstruction secondary to mesenteric desmoid formation
• Plain films may demonstrate visceral displacement or obstruction
• US useful in characterizing abdominal wall desmoid extent
• Abdominal pelvic CT scan or MRI will demonstrate a soft-tissue mass that is radiographically indistinguishable from a soft-tissue sarcoma
• Complete history and physical exam
• Radiographic characterization of tumor extent
• Core needle biopsy or incisional biopsy to establish diagnosis
• Resect desmoid tumor to a histologically negative margin
• Re-excision or radiation therapy for positive or close to positive margins
• Abdominal wall reconstruction with avoidance of alloplastic materials if possible
• Mesenteric desmoids associated with FAP are managed conservatively for as long as possible
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