title: Sporadic hemangioblastoma of the kidney with PAX2 and focal CD10 expression: report of a case.
abstract: In this study, we presented an additional case of renal hemangioblastoma, which demonstrates PAX2 and focal CD10 expression. Histologically, the tumor consisted of sheets of oval or polygonal cells and a prominent vascular network. The tumor cells varied in size, and possessed pale or eosinophilic cytoplasm that sometimes contained sharply delineated fine vacuoles. The tumor cell nuclei with inconspicuous nucleoli showed moderate nuclear atypia and pleomorphism. Focal areas of stromal hyalinization and sclerosis were detected. On account of its strong or moderate immunoreactivity for the a-inhibin, S100, NSE, and EGFR, the diagnosis of renal hemangioblastoma was established. For further evidence of VHL deficiency, the tumor was subjected to VHL sequence analysis of all three exons and fluorescence in situ hybridization (FISH) detection for chromosome 3p deletion. None of the VHL gene mutations and chromosome 3p deletion was detected in the tumor. Because of several shared morphological and immunophenotypic features, renal hemangioblastoma may be underrecognized and should be included in the differential diagnosis of primary renal tumors, in particular clear cell renal cell carcinoma. The unexpected positive staining of PAX2 and CD10 in renal hemangioblastoma should be particular concerned. Using a combination of immunoprofile may be helpful to the differential diagnosis of these renal tumors.
authors.affiliation: Department of Pathology, Jinhu County People's Hospital Jiangsu Province, China.
authors.forename: Jin-Gui
authors.initials: JG
authors.lastname: Jiang
authors.forename: Qiu
authors.initials: Q
authors.lastname: Rao
authors.forename: Qiu-Yuan
authors.initials: QY
authors.lastname: Xia
authors.forename: Pin
authors.initials: P
authors.lastname: Tu
authors.forename: Zhen-Feng
authors.initials: ZF
authors.lastname: Lu
authors.forename: Qin
authors.initials: Q
authors.lastname: Shen
authors.forename: Ru-Song
authors.initials: RS
authors.lastname: Zhang
authors.forename: Bo
authors.initials: B
authors.lastname: Yu
authors.forename: Xiao-Jun
authors.initials: XJ
authors.lastname: Zhou
authors.forename: Shan-Shan
authors.initials: SS
authors.lastname: Shi
authors.forename: Qun-Li
authors.initials: QL
authors.lastname: Shi
mesh_terms: D014408:Biomarkers, Tumor / Q000032:analysis* / Q000235:genetics; D001706:Biopsy; D002872:Chromosome Deletion; D002893:Chromosomes, Human, Pair 3; D003937:Diagnosis, Differential; D005260:Female; D018325:Hemangioblastoma / Q000737:chemistry* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D017404:In Situ Hybridization, Fluorescence; D007680:Kidney Neoplasms / Q000737:chemistry* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D009154:Mutation; D009392:Nephrectomy; D015260:Neprilysin / Q000032:analysis*; D051762:PAX2 Transcription Factor / Q000032:analysis*; D011237:Predictive Value of Tests; D016896:Treatment Outcome; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics
chemical_list: D014408:Biomarkers, Tumor; D051762:PAX2 Transcription Factor; C497318:PAX2 protein, human; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D015260:Neprilysin; C497446:VHL protein, human
keywords: CD10; Kidney; PAX2; differential diagnosis; fluorescence in situ ridization (FISH); hemangioblastoma; molecular genetics; von-Hippel-Lindau (VHL)
references.citation: Ann Diagn Pathol. 2010 Oct;14(5):361-4
references.pmid: 20850701
references.citation: Histopathology. 2013 Mar;62(4):642-50
references.pmid: 23379905
references.citation: Diagn Pathol. 2012 Apr 12;7:39
references.pmid: 22497861
references.citation: Endocr Pathol. 2012 Sep;23(3):187-90
references.pmid: 22544391
references.citation: Am J Surg Pathol. 2007 Oct;31(10):1545-51
references.pmid: 17895756
references.citation: Am J Surg Pathol. 2011 Apr;35(4):623-4
references.pmid: 21378542
references.citation: Diagn Pathol. 2012 May 01;7:49
references.pmid: 22548972
references.citation: Oncol Lett. 2013 Jan;5(1):360-362
references.pmid: 23255949
references.citation: Am J Surg Pathol. 2003 Aug;27(8):1152-6
references.pmid: 12883249
references.citation: Am J Surg Pathol. 2011 Feb;35(2):262-7
references.pmid: 21263247
references.citation: Mod Pathol. 2011 Sep;24(9):1207-20
references.pmid: 21602815
references.citation: Mod Pathol. 2005 Jun;18(6):788-94
references.pmid: 15578072
references.citation: Int J Surg Pathol. 2011 Apr;19(2):170-9
references.pmid: 20034980
references.citation: Am J Surg Pathol. 2008 Aug;32(8):1239-45
references.pmid: 18594469
references.citation: Int J Surg Pathol. 2012 Oct;20(5):519-22
references.pmid: 22271882
references.citation: Am J Surg Pathol. 2010 Nov;34(11):1695-700
references.pmid: 20924277
references.citation: Am J Surg Pathol. 2012 Sep;36(9):1327-38
references.pmid: 22895266
references.citation: Am J Surg Pathol. 2008 Dec;32(12):1780-6
references.pmid: 18779729