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PubMed1977

[Familial multiple hemangioblastomas--report of two cases in a family (author's transl)].

The authors reported their experience with two cases in a kindred with multiple hemangioblastomas in the cerebellum, brain stem, and spinal cord associated with von Hippel's disease, von Recklinghausen's disease and some visceral changes. (The members of this kindred died of similar disease.) Two patients, a 42-year-old man and his 14-year-old niece, showed various neurological signs involving central nervous system. Furthermore the man had some café-au-lait spots in the skin, a papillomatous tumors in the tongue and a vascular tumor in the right kidney. Both also had von Hippel's disease. Neuroradiological study and operations demonstrated multiple hemangioblastomas in the cerebellum, medulla oblongata and the spinal cord in association with abnormal enlargements of the medulla and the spinal cord which suggested coexistence of the parenchymatous tumor, probably glioma, in them. All hemangioblastomas in the cerebellum of both patients were removed. The literature of the familial multiple hemangioblastomas was reviewed and the coexistence of Lindau's disease, von Recklinghausen's disease and glioma was discussed.

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