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Int J Clin Exp Pathol 2013;6(9):1953-1956
Sporadic hemangioblastoma of the kidney with PAX2 and focal CD10 expression: report
of a case
Jin-Gui Jiang, Qiu Rao, Qiu-Yuan Xia, Pin Tu, Zhen-Feng Lu, Qin Shen, Ru-Song Zhang, Bo Yu, Xiao-Jun Zhou, Shan-Shan Shi, Qun-Li Shi
Department of Pathology, Jinhu County People’s Hospital, Jiangsu Province, China; Department of Pathology, Nanjing Jinling Hospital, Nanjing
University School of Medicine, Nanjing, China. Equal contributors.
Received June 19, 2013; Accepted July 12, 2013; Epub August 15, 2013; Published September 1, 2013
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. (IJCEP1306015).
Keywords: Kidney, hemangioblastoma, PAX2, CD10, von-Hippel-Lindau (VHL), fluorescence in situ ridization (FISH), molecular genetics,
Address correspondence to: Dr. Qun-Li Shi, Department of Pathology, Nanjing Jinling Hospital, Nanjing University School of Medicine,
Nanjing, China. Tel: 86-25-80860191; Fax: 86-25-80860191; E-mail: email@example.com