title: Malignant perivascular epithelioid cell tumor of the mesentery: a case report and literature review.
abstract: BACKGROUND
  Perivascular epithelioid cell tumors (PEComas) are very rare mesenchymal neoplasms, and have been found in various organs such as the liver, kidney, falciform ligament, uterus, uterine cervix, and both the small and large bowel. However, only 3 cases of mesenteric PEComa have been described in the literature so far. The treatment and prognosis of malignant mesenteric PEComas are discussed.
  
  
  METHODS
  We report the case of a 59-year-old man diagnosed with PEComa. He underwent segmental resection of the jejunum and tumor resection. Malignant mesenteric PEComa was confirmed on the basis of clinicopathological features. Tumor resection was followed by concurrent chemoradiotherapy.
  
  
  CONCLUSIONS
  Besides surgery, no effective treatment for malignant PEComa has been established thus far because of the rarity of this tumor. Here, we report our experience of treating a malignant mesenteric PEComa using surgery and subsequent adjuvant therapy, which effectively controlled disease progression and prevented local recurrence.
authors.affiliation: Department of Surgery, Division of General Surgery, Taipei, Taiwan, Republic of China.
authors.forename: Chien-Liang
authors.initials: CL
authors.lastname: Lai
authors.forename: Kuo-Feng
authors.initials: KF
authors.lastname: Hsu
authors.forename: Jyh-Cherng
authors.initials: JC
authors.lastname: Yu
authors.forename: Cheng-Jueng
authors.initials: CJ
authors.lastname: Chen
authors.forename: Chung-Bao
authors.initials: CB
authors.lastname: Hsieh
authors.forename: De-Chuan
authors.initials: DC
authors.lastname: Chan
authors.forename: Heng-Sheng
authors.initials: HS
authors.lastname: Li
authors.forename: Hung-Ming
authors.initials: HM
authors.lastname: Hsu
mesh_terms: D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D059248:Chemoradiotherapy; D003131:Combined Modality Therapy; D003937:Diagnosis, Differential; D015622:Epithelioid Cells / Q000473:pathology*; D005500:Follow-Up Studies; D006801:Humans; D007580:Jejunal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D008297:Male; D008643:Mesentery / Q000473:pathology*; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000188:drug therapy; D010534:Peritoneal Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis* / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery; D016879:Salvage Therapy; D014057:Tomography, X-Ray Computed