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    Adult Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Stage IV Adult Soft Tissue Sarcoma

    Refer to the Treatment Option Overview section of this summary for a more detailed discussion of the roles of surgery, radiation therapy, and chemotherapy.

    In the setting of lung metastasis, resection of metastatic tumors may be associated with long-term disease-free survival in patients selected for optimal underlying disease biology (i.e., patients with a limited number of metastases and slow tumor growth).[1,2,3] It is not clear to what degree the favorable outcomes are attributable to the efficacy of surgery or to careful selection of patients based upon factors that are associated with less-virulent disease.[1,2,3] The value of resection of hepatic metastases is unclear.

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    General Information About Gastrointestinal Carcinoid Tumors

    Epidemiology The age-adjusted incidence of carcinoid tumors worldwide is approximately 2 per 100,000 persons.[1,2] The average age at diagnosis is 61.4 years.[3] Carcinoid tumors represent about 0.5% of all newly diagnosed malignancies.[2,3] Anatomy Carcinoid tumors are rare, slow-growing tumors that originate in cells of the diffuse neuroendocrine system. They occur most frequently in tissues derived from the embryonic gut. Foregut tumors, which account for up to 25% of cases, arise...

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    As noted in the Treatment Option Overview section above, doxorubicin is the standard systemic therapy in the management of metastatic sarcomas.[4,5] Other drugs that are felt to have clinical activity as single agents are ifosfamide, epirubicin, gemcitabine, and paclitaxel.[6,7,8,9] Their clinical activity relative to single-agent doxorubicin is not clear, and they are not known to have superior activity. There is controversy about whether adding drugs to doxorubicin offers clinical benefit beyond what is achieved by doxorubicin as a single agent. For older patients to avoid severe toxicity, sequential use of single agents may be the preferred strategy for palliation.

    Standard treatment options

    1. Chemotherapy.
      • Single-agent chemotherapy, with subsequent single agents for disease regrowth.[4,5,6,8,9,10] Doxorubicin is generally the first-line agent. Ifosfamide also has substantial single-agent activity.
      • Doxorubicin-based combination chemotherapy. A variety of regimens have been used, but none has been proven to increase overall survival compared to doxorubicin alone.[4,5] There is some evidence that the addition of ifosfamide increases response rates (but not survival). Toxicity is increased with the addition of drugs to doxorubicin. No quality-of-life studies have been reported in comparisons of single-agent therapy versus combination therapy.
    2. Resection of pulmonary lesions may be performed if the primary tumor is under control.[1,2,3]

    Current Clinical Trials

    Check for U.S. clinical trials from NCI's list of cancer clinical trials that are now accepting patients with stage IV adult soft tissue sarcoma. The list of clinical trials can be further narrowed by location, drug, intervention, and other criteria.

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