| Abstract: |
Approximately 2400 people were diagnosed with leiomyosarcoma in 2004 in the US. This rare tumor of connective tissue is clinically subdivided into uterine and nonuterine leiomyosarcoma. No predisposing factors are known, and presentation is usually related to mass involvement and intrusion of adjacent structures. Tissue obtained for the initial diagnosis should be of sufficient quantity to evaluate the grade of the tumor, and a full work-up for the extent of disease must include chest, abdomen, and pelvic imaging. For localized disease, the standard of care remains limited to surgical excision. Radiation therapy can be considered for larger tumors and/or positive margins if anatomically feasible. Adjuvant chemotherapy has not been proven to be effective and remains investigational. Treatment for metastatic disease is palliative. Active agents include doxorubicin, ifosfamide, gemcitabine, and docetaxel. The last two agents in combination have recently been shown to be highly active in uterine leiomyosarcoma in the metastatic setting and are now being further pursued at the Memorial Sloan-Kettering Cancer Center (New York, NY, USA) in both the adjuvant uterine leiomyosarcoma setting and in the nonuterine leiomyosarcoma metastatic setting. Patients diagnosed with leiomyosarcoma should be referred to specialty sarcoma centers and encouraged to participate in clinical trials. © 2006 Adis Data Information BV. All rights reserved. |
