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  • • 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)

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Epidemiology

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  • • 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

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Symptoms and Signs

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  • • 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

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Laboratory Findings

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  • • No specific abnormalities

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Imaging Findings

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  • • 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

    • -Both methods will localize and characterize extent of the lesion

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  • • Soft-tissue sarcoma

    • Rectus sheath hematoma

    • Benign abdominal wall masses

    • -Lipoma

      -Hemangioma

      -Fibroma

      -Endometrioma

    • Abdominal wall metastasis

    • Mesenteric metastasis

    • Mesenteric cyst

    • Interloop abscess

    • Mesenteric hematoma

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Rule Out

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  • • Soft-tissue sarcoma

    • Abdominal wall metastases

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  • • Complete history and physical exam

    • Radiographic characterization of tumor extent

    • Core needle biopsy or incisional biopsy to establish diagnosis

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When to Admit

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  • • Abdominal wall lesions usually can be managed on an outpatient basis

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When to Refer

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  • • Incompletely excised lesions with microscopically positive margins may benefit from postoperative radiation

    • Most patients can be treated by the general surgeon

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  • • 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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Surgery

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Indications

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  • • Resectable abdominal wall desmoid tumor

    • Bowel obstruction secondary to desmoid that does not respond to conservative treatment

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Contraindications

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  • • Avoid resection of major neurovascular structures or adjacent organs unless absolutely necessary

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Medications

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  • • Tamoxifen

    • Sulindac

    • Chemotherapy (last resort)

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Treatment Monitoring

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  • • Excision site recurrence by physical examination or imaging

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Complications

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  • • ...

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