[Multiple malignant intracerebral schwannomas in von Recklinghausen's disease--report of a case].

Intracerebral schwannoma is very rare, and only nine cases of solitary intracerebral schwannoma have been reported since the first description by Gibson et al, in 1966. This report is the first case of multiple intracerebral schwannomas in Japan. Intracranial extracerebral schwannomas in von Recklinghausen's disease are sometimes encountered, but intracerebral ones have not been discovered. A case of multiple intracerebral schwannomas associated with von Recklinghausen's disease is reported in a 23-year-old male. He had been healthy until January 1979 when he was pointed out an abnormal shadow on chest roentgenogram. Although thoracic tumor revealed schwannoma in histological examination of surgical specimen, it transformed to malignant one during next 15 months. In July 1980, he complained of severe headache, vertigo, nausea and vomiting, and bilateral papilledema was proved. He was admitted to our clinic on August 8, 1980. Two large intracerebellar and four intracerebral tumors were revealed by computed tomography (CT). Angiography demonstrated avascular masses. The cerebellar tumors were totally extirpated on August 12, 1980. Histological diagnosis of the removed tumors approved malignant schwannoma, but their correlation to the chest tumor was unclear. Follow-up CT scans demonstrated that the supratentorial tumors increased in size during about three months. The chest tumor also increases in size. He died on November 12, 1980, but autopsy was not done. Pathogenesis of intracerebral schwannomas in this case is controversial, and we suggest that it is (1) multiple schwannomas in von Recklinghausen's disease with malignant transformation, or (2) metastasis from the chest tumor. But the conclusion remains unclear.