Cerebral Hemangioblastoma Occurring in a Patient with von Hippel-Lindau Disease

s INCE Pye-Smith's first report in 1885, la a variety of pathological entities commonly found in patients with cerebellar hemangioblastomas have been described. Spinal cord hemangioblastoma, angiomatosis retinae, polycystic kidneys, pancreatic cysts, hypernephroma, and erythrocytosis are among the associated lesions reported? -7,u,17,21 The retinal lesions were described by von Hippel in 1904. 9 Later Lindau described the syndrome which included retinal and cerebellar neoplasms and noted the frequent occurrence of the disease in families. 1~ Subsequently, the familial pattern of the syndrome was confirmed by others. The occurrence of cerebral hemangioblastoma in a patient with von HippelLindau disease is very rare. In 1931, Rochat described a cerebral hemangioblastoma unassociated with a cyst occurring in a 20-yearman with multiple cerebellar hemangioblastomas and a family history of angiomatosisY Since then, occasional angioblastic tumors arising in the cerebrum have been documented, but none associated with angiomas elsewhere in the body. 2-4,1~176 We are reporting a case in which a cerebral hemangioblastoma occurred in a patient with von Hippel-Lindau disease.