Von Hippel-Lindau病の2症例

概要

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Two cases of von Hippel-Lindau disease are presented. Case 1 was a 52-year-old male with four lesions: a cerebellar hemangioblastoma, an intraventricular metastatic tumor, a renal cell carcinoma, and a liver cyst. The patient died of intraventricular hemorrhage due to a metastatic brain tumor of the renal cell carcinoma. Brain metastasis of the renal cell carcinoma of von Hippel-Lindau disease is extremely rare. Case 2 was a 24-year-old male with five lesions: a cerebellar hemangioblastoma, a spinal hemangioblastoma, a pancreatic cyst, a pheochromocytoma, and multiple renal cysts. Spinal angiography was useful in the diagnosis of the spinal tumor. The elder brother of the patient has an asymptomatic renal cyst. Twenty-five distinct lesions have been reported in von Hippel-Lindau disease. Computed tomographic screening seems to provide typical image of the central nervous and abdominal tumors.
日本脳神経外科学会の論文
1986-10-15