Sporadic Hemangioblastoma of the Kidney: a rare renal tumor

Yang Liu,Xue-Shan Qiu,En-Hua Wang

doi:10.1186/1746-1596-7-49

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

Highlights

Hemangioblastoma, known as capillary hemangioblastoma, is a benign tumor of uncertain histogenesis, consisting of networks of small blood vessels interspersed with lipid-laden stromal cells [1]
We report one such case involving the kidney, which might be mistaken for other renal tumors, in particular clear cell renal cell carcinoma
A correct diagnosis is important because hemangioblastoma is benign even if there are highly atypical tumor cells, and the patient should be evaluated for possible von Hippel-Lindau disease

Summary

Background

Hemangioblastoma, known as capillary hemangioblastoma, is a benign tumor of uncertain histogenesis, consisting of networks of small blood vessels interspersed with lipid-laden stromal cells [1]. Hemangioblastoma occurs sporadically, or in a setting of von Hippel-Lindau disease in approximately one-quarter of the cases [2]. This tumor typically occurs within the central nervous system, predominantly in the cerebellum, and occasionally in the meninges, retina, spinal cord, corpus callosum, lateral ventricle, pituitary gland, and the optic nerve. The cellular areas were composed of a rich capillary network of single-layered flat endothelial cells enclosing stromal cells. These last ones showed a pale or eosinophilic cytoplasm exhibiting occasional lipid droplets but no hyaline globules, ovalnuclei, delicate chromatin, and inconspicuous nucleoli (Figure 1F-G).

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