In three brothers of a family with a high incidence of cancer a bilateral renal-cell carcinoma was demonstrated. In the index case there was also a cerebellar haemangioblastoma. At necropsy primary bilateral renal-cell carcinoma was demonstrated while intrarenal metastases were excluded. The cases are considered to be predominantly renal manifestations of the von Hippel-Lindau syndrome with facultative oculocerebellar involvement. Urological and cytogenetic study of other family members provided no evidence of further manifestations of the syndrome. Clinical supervision of members of such families is indicated.

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