title: Sporadic hemangioblastoma of the kidney in a 29-year-old man.
abstract: Hemangioblastoma 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.
authors.affiliation: National Taiwan University Hospital, Taipei, Taiwan.
authors.forename: Chung-Chieh
authors.initials: CC
authors.lastname: Wang
authors.forename: Shuo-Meng
authors.initials: SM
authors.lastname: Wang
authors.forename: Jau-Yu
authors.initials: JY
authors.lastname: Liau
mesh_terms: D000328:Adult; D014408:Biomarkers, Tumor / Q000378:metabolism; D002292:Carcinoma, Renal Cell / Q000175:diagnosis; D003937:Diagnosis, Differential; D018325:Hemangioblastoma / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D007265:Inhibins / Q000378:metabolism; D007680:Kidney Neoplasms / Q000378:metabolism / Q000473:pathology*; D008297:Male; D010751:Phosphopyruvate Hydratase / Q000378:metabolism; D035583:Rare Diseases; D009418:S100 Proteins / Q000378:metabolism; D006623:von Hippel-Lindau Disease / Q000175:diagnosis
chemical_list: D014408:Biomarkers, Tumor; D009418:S100 Proteins; C094377:inhibin-alpha subunit; D007265:Inhibins; D010751:Phosphopyruvate Hydratase