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  • • 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

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Epidemiology

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  • • 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

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Symptoms and Signs

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  • • 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

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Imaging Findings

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  • 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

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  • • Soft-tissue sarcoma

    • Traumatic injury

    • Hematoma

    • Cutaneous neoplasm/metastasis

    • Desmoid tumor

    • Subcutaneous or intramuscular lipoma

    • Abscess

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Rule Out

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  • • Other cutaneous malignancy or metastasis

    • Hematoma

    • Abscess

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  • • 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

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When to Admit

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  • • Extremity sarcoma work-up can be performed as an outpatient

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When to Refer

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  • • A multidisciplinary approach is essential to ensure optimal outcomes, including an oncology surgeon, medical oncology, and radiation-oncology

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  • • 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

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Surgery

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Indications

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  • • Initial incisional biopsy to establish diagnosis (always orientate extremity incision longitudinally)

    • Therapeutic wide local excision, ideally with 2 cm margin vs amputation in patients whose tumors cannot be resected ...

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