Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • 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) +++ Epidemiology + • 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 +++ Symptoms and Signs + • 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 +++ Laboratory Findings + • No specific abnormalities +++ Imaging Findings + • 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 + • 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 +++ Rule Out + • Soft-tissue sarcoma• Abdominal wall metastases + • Complete history and physical exam• Radiographic characterization of tumor extent• Core needle biopsy or incisional biopsy to establish diagnosis +++ When to Admit + • Abdominal wall lesions usually can be managed on an outpatient basis +++ When to Refer + • Incompletely excised lesions with microscopically positive margins may benefit from postoperative radiation• Most patients can be treated by the general surgeon + • 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 +++ Surgery +++ Indications + • Resectable abdominal wall desmoid tumor• Bowel obstruction secondary to desmoid that does not respond to conservative treatment +++ Contraindications + • Avoid resection of major neurovascular structures or adjacent organs unless absolutely necessary +++ Medications + • Tamoxifen• Sulindac• Chemotherapy (last resort) +++ Treatment Monitoring + • Excision site recurrence by ... Your Access profile is currently affiliated with [InstitutionA] and is in the process of switching affiliations to [InstitutionB]. Please select how you would like to proceed. Keep the current affiliation with [InstitutionA] and continue with the Access profile sign in process Switch affiliation to [InstitutionB] and continue with the Access profile sign in process Get Free Access Through Your Institution Learn how to see if your library subscribes to McGraw Hill Medical products. Subscribe: Institutional or Individual Sign In Error: Incorrect UserName or Password Username Error: Please enter User Name Password Error: Please enter Password Sign in Forgot Password? Forgot Username? Download the Access App: iOS | Android Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free profile for additional features.