Sections View Full Chapter Figures Tables Videos Annotate Full Chapter Figures Tables Videos Supplementary Content + • Soft-tissue sarcomas present as asymptomatic large masses in the extremities or retroperitoneum but may also develop occasionally in the neck or within the abdominal viscera• Originate from a wide variety of mesenchymal cell types and include:-Liposarcoma-Fibrosarcoma-Rhabdomyosarcoma-Leiomyosarcoma-Desmoid tumors• Tumor grade rather than the cell of origin determines natural history• Most soft-tissue sarcomas arise de novo; rarely result from malignant degeneration of a benign lesion• Sarcomas generally metastasize via a hematogenous route, most commonly to the lung• Most important prognostic factors include tumor size and grade +++ Epidemiology + • Account for 1% of all new cancer diagnoses• 50% of soft-tissue sarcomas arise in the lower extremities, most commonly in the thigh• There are familial syndromes that genetically predispose patients to the formation of soft-tissue sarcomas:-Gardner syndrome: Desmoid tumors-Recklinghausen disease: Neurofibrosarcomas-Li Fraumeni syndrome• Risk factors include:-Irradiation-Chronic extremity lymphedema +++ Symptoms and Signs + • Asymptomatic large soft-tissue mass• Nerve compression/invasion may result in pain, parasthesias, or neuropathy• Venous compression/invasion may result in deep venous thrombosis (DVT) formation +++ Imaging Findings + • Chest x-ray: May demonstrate evidence of metastases• CT scan: Demonstrates a homogenous soft-tissue density mass and differentiate between hematoma or abscess• MRI-Accurately defines the extent of the sarcoma and invasion of surrounding structures-Probably the best imaging test in the evaluation of an extremity sarcoma + • Soft-tissue sarcoma• Traumatic injury• Hematoma• Cutaneous neoplasm/metastasis• Desmoid tumor• Subcutaneous or intramuscular lipoma• Abscess +++ Rule Out + • Other cutaneous malignancy or metastasis• Hematoma• Abscess + • Thorough history and physical exam• Core needle or incisional biopsy to establish diagnosis• CT or MRI (preferred) to define extent of tumor and invasion of surrounding structures• Chest x-ray or CT to evaluate for evidence of pulmonary metastases +++ When to Admit + • Extremity sarcoma work-up can be performed as an outpatient +++ When to Refer + • A multidisciplinary approach is essential to ensure optimal outcomes, including an oncology surgeon, medical oncology, and radiation-oncology + • Establish diagnosis histologically with a core needle biopsy or incisional biopsy• Radiographically define extent of tumor and invasion of surrounding structures• Evaluate for pulmonary metastases• Surgical wide local excision with a 2 cm margin• Postoperative radiation therapy for all high-grade sarcomas and those > 2 cm• Sometimes adjuvant chemotherapy• Preoperative radiation therapy for patients with tumors larger than 10 cm or to facilitate limb-sparing procedures +++ Surgery +++ Indications + • Initial incisional biopsy to establish diagnosis (always orientate extremity incision longitudinally)• Therapeutic wide local excision, ideally with 2 cm margin vs ... Your Access profile is currently affiliated with '[InstitutionA]' and is in the process of switching affiliations to '[InstitutionB]'. Please click ‘Continue’ to continue the affiliation switch, otherwise click ‘Cancel’ to cancel signing in. 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 Username Error: Please enter User Name Password Error: Please enter Password Forgot Password? Forgot Username? Sign in via OpenAthens Sign in via Shibboleth You already have access! Please proceed to your institution's subscription. Create a free a profile for additional features.