Wang C.-C.SHUO-MENG WANGJAU-YU LIAU2020-03-052020-03-0520121066-8969https://www.scopus.com/inward/record.uri?eid=2-s2.0-84866675319&doi=10.1177%2f1066896911434548&partnerID=40&md5=fb97be9897c72c1245082df0e7ce7f95https://scholars.lib.ntu.edu.tw/handle/123456789/470375Hemangioblastoma of the kidney is a rare, newly recognized tumor with morphological features similar to its cerebellar counterpart. There have been only 4 cases reported in the literature in English, all of them occurring in middle-aged to elderly patients. Here, we report a case of renal hemangioblastoma in a young adult without clinical evidence of von Hippel-Lindau disease. The tumor was composed of polygonal cells with mildly eosinophilic to clear cytoplasm and a rich vascular network. Immunohistochemical staining revealed a typical profile (positivity for α-inhibin, neuron-specific enolase and S100; negative results for epithelial membrane antigen, HMB-45, and Melan-A), which confirmed the diagnosis. Despite the similarity to renal-cell carcinoma in morphology, hemangioblastoma of the kidney is clinically indolent. Correct recognition of this pathological entity is important to avoid overdiagnosis and unnecessary clinical treatment. ? The Author(s) 2012.[SDGs]SDG3alpha inhibin; inhibin; neuron specific enolase; protein S 100; unclassified drug; adult; article; case report; computer assisted tomography; echography; hemangioblastoma; histopathology; human; human tissue; immunohistochemistry; kidney tumor; male; nephrectomy; nuclear magnetic resonance imaging; priority journal; Adult; Carcinoma, Renal Cell; Diagnosis, Differential; Hemangioblastoma; Humans; Inhibins; Kidney Neoplasms; Male; Phosphopyruvate Hydratase; Rare Diseases; S100 Proteins; Tumor Markers, Biological; von Hippel-Lindau Diseasejournal article10.1177/1066896911434548222718822-s2.0-84866675319