Metastasis of renal cell carcinoma to a haemangioblastoma of the medulla oblongata in von Hippel-Lindau syndrome.

We describe a patient with von Hippel-Lindau (VHL) syndrome with metastasis of renal cell carcinoma (RCC) to a haemangioblastoma (HAB) of the medulla oblongata. Histological examination revealed cells with clear cytoplasm arranged in alveolar or tubular patterns in most fields of view. In a small area, the tumor showed abundant thin-walled vessels and clear cells. Immunohistochemically, staining for cytokeratin, epithelial membrane antigen and CD10 was typically positive in the tumor portion that contained RCC but not in the HAB. The HAB was immunopositive for inhibin-alpha and CD34. Detailed histological analysis and immunohistochemistry should allow differentiation of HAB and RCC. Copyright 2010 Elsevier Ltd. All rights reserved.