Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang 110001, China.
Diagn Pathol. 2012 May 1;7:49. doi: 10.1186/1746-1596-7-49.
Hemangioblastoma is a benign and morphologically distinctive tumor that can occur sporadically or in association with von Hippel-Lindau disease in approximately 25% of the cases, and which involves the central nervous system in the majority of the cases. Rare occurrences of hemangioblastoma in peripheral nerves and extraneural tissues have been reported. This report describes one case of sporadic renal hemangioblastoma happened in a 16-year-old Chinese female patient, presenting with hematuria, and low back pain. Histologically, the tumors were circumscribed, and composed of sheets of large polygonal cells traversed by arborizing thin-walled blood vessels. The diagnosis of hemangioblastoma was confirmed by negative immunostaining for cytokeratin, and positive staining for α-inhibin, S100 and neuron-specific enolase (NSE). This benign neoplasm which can be mistaken for various malignancies such as renal cell carcinoma, epithelioid hemangiopericytoma and epithelioid angiomyolipoma, deserves wider recognition for its occurrence as a primary renal tumor.
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血管母细胞瘤是一种良性的、形态独特的肿瘤,约 25%的病例为散发性,25%的病例与 von Hippel-Lindau 病有关,且多数发生于中枢神经系统。偶有血管母细胞瘤发生于外周神经和神经外组织的报道。本报告描述了一例散发性肾血管母细胞瘤病例,患者为 16 岁中国女性,表现为血尿和腰痛。组织学上,肿瘤边界清楚,由多边形细胞构成,细胞间穿行有树枝状薄壁血管。免疫组织化学染色阴性表达细胞角蛋白,阳性表达α-抑制素、S100 和神经元特异性烯醇化酶(NSE),有助于明确诊断为血管母细胞瘤。该良性肿瘤可能被误诊为各种恶性肿瘤,如肾细胞癌、上皮样血管外皮细胞瘤和上皮样血管平滑肌脂肪瘤,因其作为原发性肾肿瘤而值得广泛认识。
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