title: [Clinicopathologic study of primary renal hemangioblastoma].
abstract: OBJECTIVE
  To study the clinicopathologic characteristics of primary renal hemangioblastoma.
  
  
  METHODS
  The morphologic features, immunophenotype and molecular findings of 3 cases of primary renal hemangioblastoma were studied, with review of literature.
  
  
  RESULTS
  The age of patients ranged from 43 to 57 years. There were 2 women and a man. The patients often presented with renal mass. Histologically, the tumors were surrounded by thick fibrous capsule and composed of epithelioid or spindle cells. Two cases had a prominent stromal component and the other one was rich in capillary network. Lipid vacuoles were observed in all cases. Features of hemorrhage were demonstrated in 2 cases. Capsular invasion and necrosis were seen in 1 case. Immunohistochemical study showed that the stromal cells were positive for alpha-inhibin (3/3), S-100 protein (3/3), EGFR (2/2), PAX-2 (2/2), PAX-8 (2/2) and CA9 (2/2) but negative for CKpan (2/2) and HMB45 (2/2). Focal membranous staining for CD10 (3/3) was noted. No VHL gene mutations or chromosome 3p deletion were detected in the 2 cases studied.
  
  
  CONCLUSIONS
  Renal hemangioblastoma shows distinctive morphologic appearance with a wide range of variation. The unexpected positive staining for PAX-2, PAX-8 and CD10 in renal hemangioblastoma needs to be aware. Immunohistochemical study may be helpful in differential diagnosis of these renal tumors.
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Xueping
authors.initials: X
authors.lastname: Zhang
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Xuan
authors.initials: X
authors.lastname: Wang
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Jianjun
authors.initials: J
authors.lastname: Wang
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Qin
authors.initials: Q
authors.lastname: Shen
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Qiuyuan
authors.initials: Q
authors.lastname: Xia
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Shanshan
authors.initials: S
authors.lastname: Shi
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Zhenfeng
authors.initials: Z
authors.lastname: Lu
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Henghui
authors.initials: H
authors.lastname: Ma
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Rusong
authors.initials: R
authors.lastname: Zhang
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Yan
authors.initials: Y
authors.lastname: He
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Bo
authors.initials: B
authors.lastname: Yu
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China.
authors.forename: Xiaojun
authors.initials: X
authors.lastname: Zhou
authors.affiliation: Department of Pathology, Medicine School of Nanjing University/Nanjing Jinling Hospital, Nanjing 210002, China; E-mail: raoqiu1103@126.com.
authors.forename: Qiu
authors.initials: Q
authors.lastname: Rao
mesh_terms: D000328:Adult; D002872:Chromosome Deletion; D002893:Chromosomes, Human, Pair 3; D003937:Diagnosis, Differential; D005260:Female; D018325:Hemangioblastoma / Q000737:chemistry / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D016130:Immunophenotyping; D007265:Inhibins / Q000032:analysis; D007680:Kidney Neoplasms / Q000737:chemistry / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins / Q000032:analysis; D015260:Neprilysin / Q000032:analysis; D051762:PAX2 Transcription Factor / Q000032:analysis; D000072636:PAX8 Transcription Factor / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D017154:Stromal Cells / Q000737:chemistry
chemical_list: D009363:Neoplasm Proteins; D051762:PAX2 Transcription Factor; C497318:PAX2 protein, human; D000072636:PAX8 Transcription Factor; C083325:PAX8 protein, human; D009418:S100 Proteins; C094377:inhibin-alpha subunit; D007265:Inhibins; D015260:Neprilysin