title: Case report of extrarenal rhabdoid tumor of pelvic retroperitoneum molecular profile of angiogenesis and its implication in new treatment strategy.
abstract: We report the detailed molecular study of angiogenesis-ralated genes and target therapy of the case of a male 46-year-old patient with extrarenal rhabdoid tumor of pelvic retroperitoneum. The patient was found to have a huge pelvic soft tissue sarcoma and underwent pelvic tumorectomy and appendectomy. The microscopically morphological features and molecular profile by immunohistochemical analysis supported the surgical histological diagnosis of extrarenal rhabdoid tumor. The tumor recurred two weeks after surgery and metastasized to the lung, left abdominal wall and mesenteric lymph nodes. Systemic chemotherapy including ifosfamide, liposomal doxorubicin, Taxol and cisplatin, concurrently with pelvic radiotherapy (58 Gy of total dose). However, the patient did not respond to the combination of chemotherapy and radiotherapy. Immunohistochemical staining and fluorescence in situ hybridization of tumor cells indicated negative expression of human epidermal growth factor receptor-2 (HER-2) and epidermal growth factor receptor (EGFR) and positive expression of vascular endothelial growth factor (VEGF) and vascular endothelial growth factor receptor (VEGFR). So anti-VEGF targeted therapy (Bevacizumab) was administered following the fourth course chemotherapy. However, the condition worsened after the administration of the second cycle of Bevacizumab. Multiple organ failure led to the death of the patient. The patient only survived five months and 20 days after the surgery of the primary tumor.
authors.affiliation: Department of Colorectal Surgery, Cancer Hospital, Fudan University, Shanghai, China.
authors.forename: Guoxiang
authors.initials: G
authors.lastname: Cai
authors.forename: Xiaoli
authors.initials: X
authors.lastname: Zhu
authors.forename: Ye
authors.initials: Y
authors.lastname: Xu
authors.forename: Xiang
authors.initials: X
authors.lastname: Du
authors.forename: Zhen
authors.initials: Z
authors.lastname: Zhang
authors.forename: Yingjian
authors.initials: Y
authors.lastname: Zhang
authors.forename: Tongzhen
authors.initials: T
authors.lastname: Chen
authors.forename: Xiaoyan
authors.initials: X
authors.lastname: Zhou
authors.forename: Zuqing
authors.initials: Z
authors.lastname: Guan
authors.forename: Sanjun
authors.initials: S
authors.lastname: Cai
mesh_terms: D003937:Diagnosis, Differential; D017809:Fatal Outcome; D006801:Humans; D007150:Immunohistochemistry; D007633:Keratins / Q000032:analysis; D008297:Male; D008875:Middle Aged; D009389:Neovascularization, Pathologic / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D012186:Retroperitoneal Neoplasms / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000378:metabolism / Q000628:therapy; D009418:S100 Proteins / Q000032:analysis; D016879:Salvage Therapy / Q000379:methods; D014746:Vimentin / Q000032:analysis
chemical_list: D009418:S100 Proteins; D014746:Vimentin; D007633:Keratins