Peng Junjie, Yu Baohua, Wang Jian, Hong Xiaonan, Xu Ye, Cai Sanjun
Department of Abdominal, Cancer Hospital, Fudan University, Shanghai, People's Republic of China.
J Pediatr Hematol Oncol. 2010 May;32(4):e136-8. doi: 10.1097/MPH.0b013e3181b8700b.
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.
恶性血管周上皮样细胞瘤（PEComa）是一种具有恶性行为且预后较差的罕见疾病。几乎所有的PEComa对HMB45均表现出免疫活性。恶性PEComa的治疗策略仍存在争议，尤其是在晚期或不可切除的疾病中。我们报告了一名14岁不可切除的恶性PEComa患者，该患者接受了多种联合化疗。尽管使用了3种不同的联合化疗方案和抗血管生成药物，但患者在术后9个月死于肿瘤。我们的病例表明，软组织肉瘤的经验性化疗方案可能不适用于PEComa。
