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. 2013 Nov;2(4):127-9.
doi: 10.5582/irdr.2013.v2.4.127.

Leiomyosarcoma: Principles of management

Juan Martin-Liberal  1
Affiliations

Leiomyosarcoma: Principles of management

Juan Martin-Liberal. Intractable Rare Dis Res. 2013 Nov.

Abstract

The term soft-tissue sarcomas (STS) embraces more than 50 different sub-types that are often associated with poor prognosis. Only a very limited number of agents are active against STS. Doxorubicin and ifosfamide are widely accepted as the most effective compounds. However, their low response rates and poor impact on the overall survival of the patients illustrate the need for new treatment options. Among them, leiomyosarcomas are one of the most frequently occurring subtypes. In spite of the relatively high incidence of leiomyosarcomas, the overall effectiveness of the currently available systemic treatments is still poor. The heterogeneity of its biological origin, clinical behavior and responsiveness to chemotherapy, together with the scarcity of successful clinical trials, makes the treatment of leiomyosarcoma especially challenging. In addition, the evidence-based treatment for leiomyosarcoma comes from trials in which, in the majority of cases, no distinctions have been made among the different STS sub-types. As a result, every therapeutic decision should be made on an individual basis in collaboration with the patient. The results of new specific histology-designed clinical trials should aid decision making in this complex field.

Keywords: Leiomyosarcoma; chemotherapy; hormone treatment; sarcoma; targeted therapies.

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References

    1. Van Glabbeke M, van Oosterom AT, Oosterhuis JW, Mouridsen H, Crowther D, Somers R, Verweij J, Santoro A, Buesa J, Tursz T. Prognostic factors for the outcome of chemotherapy in advanced soft tissue sarcoma: An analysis of 2,185 patients treated with anthracycline-containing first-line regimens--a European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group Study. J Clin Oncol. 1999; 17:150-157 - PubMed
    1. van Oosterom AT, Mouridsen HT, Nielsen OS, Dombernowsky P, Krzemieniecki K, Judson I, Svancarova L, Spooner D, Hermans C, Van Glabbeke M, Verweij J; EORTC Soft Tissue and Bone Sarcoma Group. Results of randomised studies of the EORTC Soft Tissue and Bone Sarcoma Group (STBSG) with two different ifosfamide regimens in first- and second-line chemotherapy in advanced soft tissue sarcoma patients. Eur J Cancer. 2002; 38:2397-2406 - PubMed
    1. Leahy M, Garcia Del Muro X, Reichardt P, Judson I, Staddon A, Verweij J, Baffoe-Bonnie A, Jönsson L, Musayev A, Justo N, Burke T, Blay JY; SABINE Investigators. Chemotherapy treatment patterns and clinical outcomes in patients with metastatic soft tissue sarcoma. The SArcoma treatment and Burden of Illness in North America and Europe (SABINE) study. Ann Oncol. 2012; 23:2763-2770 - PubMed
    1. Ferrari A, Sultan I, Huang TT, Rodriguez-Galindo C, Shehadeh A, Meazza C, Ness KK, Casanova M, Spunt SL. Soft tissue sarcoma across the age spectrum: A population-based study from the Surveillance Epidemiology and End Results database. Pediatr Blood Cancer. 2011; 57:943-949 - PMC - PubMed
    1. Stiller CA, Trama A, Serraino D, Rossi S, Navarro C, Chirlaque MD, Casali PG; RARECARE Working Group. Descriptive epidemiology of sarcomas in Europe: Report from the RARECARE project. Eur J Cancer. 2013; 49:684-695 - PubMed