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Open Access Case Report

Sporadic Hemangioblastoma of the Kidney: a rare renal tumor

Yang Liu12, Xue-shan Qiu12* and En-Hua Wang12

Author Affiliations

1 Department of Pathology, the First Affiliated Hospital and College of Basic Medical Sciences, China Medical University, Shenyang, 110001, China

2 Institute of Pathology and Pathophysiology, China Medical University, Shenyang, 110001, China

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Diagnostic Pathology 2012, 7:49  doi:10.1186/1746-1596-7-49

Published: 1 May 2012

Abstract

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.

The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/5445834246942699

Keywords:
Hemangioblastoma; Von Hippel-Lindau disease; Renal cell carcinoma; Epithelioid hemangiopericytoma; Epithelioid angiomyolipoma