title: An unresectable malignant perivascular epithelioid cell tumor resistant to multiple combined chemotherapies: a unique case report.
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.
authors.affiliation: Department of Abdominal, Cancer Hospital, Fudan University, Shanghai, People's Republic of China. junjiepeng67@gmail.com
authors.forename: Junjie
authors.initials: J
authors.lastname: Peng
authors.forename: Baohua
authors.initials: B
authors.lastname: Yu
authors.forename: Jian
authors.initials: J
authors.lastname: Wang
authors.forename: Xiaonan
authors.initials: X
authors.lastname: Hong
authors.forename: Ye
authors.initials: Y
authors.lastname: Xu
authors.forename: Sanjun
authors.initials: S
authors.lastname: Cai
mesh_terms: D000293:Adolescent; D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy; D017809:Fatal Outcome; D006801:Humans; D008297:Male; D054973:Perivascular Epithelioid Cell Neoplasms / Q000188:drug therapy* / Q000601:surgery*; D016896:Treatment Outcome
chemical_list: D020533:Angiogenesis Inhibitors