PMID- 24040464 OWN - NLM STAT- MEDLINE DCOM- 20140418 LR - 20211021 IS - 1936-2625 (Electronic) IS - 1936-2625 (Linking) VI - 6 IP - 9 DP - 2013 TI - Sporadic hemangioblastoma of the kidney with PAX2 and focal CD10 expression: report of a case. PG - 1953-6 AB - 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. FAU - Jiang, Jin-Gui AU - Jiang JG AD - Department of Pathology, Jinhu County People's Hospital Jiangsu Province, China. FAU - Rao, Qiu AU - Rao Q FAU - Xia, Qiu-Yuan AU - Xia QY FAU - Tu, Pin AU - Tu P FAU - Lu, Zhen-Feng AU - Lu ZF FAU - Shen, Qin AU - Shen Q FAU - Zhang, Ru-Song AU - Zhang RS FAU - Yu, Bo AU - Yu B FAU - Zhou, Xiao-Jun AU - Zhou XJ FAU - Shi, Shan-Shan AU - Shi SS FAU - Shi, Qun-Li AU - Shi QL LA - eng PT - Case Reports PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20130815 PL - United States TA - Int J Clin Exp Pathol JT - International journal of clinical and experimental pathology JID - 101480565 RN - 0 (Biomarkers, Tumor) RN - 0 (PAX2 Transcription Factor) RN - 0 (PAX2 protein, human) RN - EC 2.3.2.27 (Von Hippel-Lindau Tumor Suppressor Protein) RN - EC 3.4.24.11 (Neprilysin) RN - EC 6.3.2.- (VHL protein, human) SB - IM MH - Biomarkers, Tumor/*analysis/genetics MH - Biopsy MH - Chromosome Deletion MH - Chromosomes, Human, Pair 3 MH - Diagnosis, Differential MH - Female MH - Hemangioblastoma/*chemistry/genetics/pathology/surgery MH - Humans MH - Immunohistochemistry MH - In Situ Hybridization, Fluorescence MH - Kidney Neoplasms/*chemistry/genetics/pathology/surgery MH - Middle Aged MH - Mutation MH - Nephrectomy MH - Neprilysin/*analysis MH - PAX2 Transcription Factor/*analysis MH - Predictive Value of Tests MH - Treatment Outcome MH - Von Hippel-Lindau Tumor Suppressor Protein/genetics PMC - PMC3759506 OTO - NOTNLM OT - CD10 OT - Kidney OT - PAX2 OT - differential diagnosis OT - fluorescence in situ ridization (FISH) OT - hemangioblastoma OT - molecular genetics OT - von-Hippel-Lindau (VHL) EDAT- 2013/09/17 06:00 MHDA- 2014/04/20 06:00 PMCR- 2013/08/15 CRDT- 2013/09/17 06:00 PHST- 2013/06/19 00:00 [received] PHST- 2013/07/12 00:00 [accepted] PHST- 2013/09/17 06:00 [entrez] PHST- 2013/09/17 06:00 [pubmed] PHST- 2014/04/20 06:00 [medline] PHST- 2013/08/15 00:00 [pmc-release] PST - epublish SO - Int J Clin Exp Pathol. 2013 Aug 15;6(9):1953-6. eCollection 2013.