[A case of multiple cerebellar hemangioblastomas with congenital deafness, juvenile diabetes mellitus and retinal angioma].

It has already been noted that hemangioblastoma is occasionally complicated with various diseases, especially retinal angioma, cysts of kidney and/or pancreas, vascular disorders, and furthermore about 10% of hemangioblastoma are multiple. The authors report here a case of multiple cerebellar hemangioblastomas accompanied with congenital deafness, juvenile diabetes mellitus and retinal angioma. The patient, a 38-year-old man who complained of disturbance of consciousness, headache and vomiting, admitted to our hospital on October 6, 1982. He was born in consanguineous marriage family and his elder sister was also suffered from congenital deafness and juvenile diabetes mellitus, but no angiomas. CT and angiography showed a left side cystic and a right side small cerebellar lesions with remarkable ventricular enlargement. Ventriculo-peritoneal shunt and suboccipital craniectomy & removal of the tumors were performed on October 6, and November 8, 1982 succeedingly. From a point of view of hereditary occurrence, the authors investigated these complicated lesions recognized in this patient. It revealed that this case did not belong to any other categories of hereditary syndromes which were already reported and so authors considered that this was an extremely rare condition. Moreover, in case of multiple hemangiomas, cerebral angiography could be a more useful method than CT scan to detect multiplicity of the lesions in the posterior fossa.