This article presents an overview of the current literature about the biology, pathology, and the clinical management of leiomyosarcoma. In addition, the article emphasizes and discusses the current systemic treatment options available for patients with leiomyosarcoma, which range from cytotoxic chemotherapy to target therapies. Particular leiomyosarcoma subtypes, such as uterine leiomyosarcoma and inferior vena cava leiomyosarcoma, are discussed separately.
Key points
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Leiomyosarcoma is a malignant mesenchymal tumor that derives from the smooth muscle lineage.
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The underlying genetic mechanisms remain unclear, and complex and unbalanced karyotypic defects are the only shared features observed across the different leiomyosarcoma subtypes.
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Cell-cycle perturbations, mainly through RB1 defects, and phosphatidylinositol 3 kinase/Akt pathway activation caused by PTEN genomic deletion are the two most consistent drivers commonly observed in sarcomas, including leiomyosarcoma.
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Patients with unresectable metastatic leiomyosarcoma are considered incurable and the treatment intention for systemic disease is always palliative.
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Unlike other soft tissue sarcomas, leiomyosarcomas are particularly sensitive to the combination of gemcitabine-docetaxel, which is currently regarded as a standard of care in this population.
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Chemotherapy treatment with trabectedin has shown exquisite activity in leiomyosarcoma, mainly in the form of long disease stabilization.
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Pazopanib is an oral multikinase inhibitor recently approved for the treatment of soft tissue sarcomas in leiomyosarcoma.
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