An Unresectable Malignant Perivascular Epithelioid Cell Tumor Resistant to Multiple Combined Chemotherapies
- 1 May 2010
- journal article
- case report
- Published by Ovid Technologies (Wolters Kluwer Health) in Journal of Pediatric Hematology/Oncology
- Vol. 32 (4), e136-e138
- https://doi.org/10.1097/mph.0b013e3181b8700b
Abstract
Malignant perivascular epithelioid cell tumor (PEComa) was a kind of rare disease with malignant behavior and poor prognosis. Nearly all PEComas show immunoactivity for HMB45. The treatment strategy for malignant PEComa is still of controversy, especially in advanced or unresectable disease. We reported a 14-year-old patient of unresectable malignant PEComa, who was treated with multiple combined chemotherapies. The patient died of tumor 9 months after surgery, although 3 different combined chemotherapeutic regimens and antiangiogenic drug were used. Our case suggested that empirical chemotherapeutic regimens for soft tissue sarcomas might not be suitable for PEComa.Keywords
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