Abstract

A rare case of familial Hippel-Lindau disease, which is an autosomal dominant disorder with inherited susceptibility to various forms of cancer, is reported in a 46-year-old father and his sons. The father complained of a malignant hypernephroma, which metastasized in the upper jaw 6 months later. As in this patient, renal cell carcinomas are fatal and are exhibited at an early age. Consequentially, patients with familial von Hippel-Lindau disease exhibiting cases of renal cell carcinoma should be examined very carefully in the head and neck region to detect and treat metastases as early as possible.

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