Abstract
Leiomyosarcoma of soft tissue is a rare tumor affecting one new patient in every 100,000 people yearly. The most frequent sites of origin are the retroperitoneum and abdomen, followed by the limbs and trunk, skin, major vessels, and, rarely, organs. Increasing evidence suggests that all of these tumors originate from the smooth cell muscles of blood vessels. Surgery is the main treatment for localized disease. The risk of disease progression at distant sites outweighs that of local recurrence, suggesting that extensive resections are not recommended, particularly for retroperitoneal tumors. Moreover, leiomyosarcomas are sensitive to various chemotherapy regimens. The eighth edition of the AJCC TNM staging system introduced new accurate nomograms for predicting patient survival that are improving prognostic stratification for identifying those patients at higher risk for disease progression who are likely to benefit from perioperative treatments. More research for this rare tumor is needed to identify innovative predicting and prognostic biomarkers and new effective treatment strategies. This review contains 14 figures, 3 tables and 56 references Key words: inferior vena cava leiomyosarcoma, leiomyosarcoma, retroperitoneal sarcoma
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