[Two cases of von Recklinghausen's disease with multiple brain and spinal tumors]

Abstract

The authors reported two cases of von Recklinghausen's disease with multiple brain tumors and multiple spinal tumors. The first case, a 21-year-old man who had a past history of optic gliomas was admitted because of gait disturbance. Computed tomography (CT) and magnetic resonance imaging (MRI) showed calcification of the basal ganglia, bilateral C-P angle tumors, cystic cerebellar tumor and arachnoid cyst in the quadrigeminal cistern. Myelography and MRI revealed multiple spinal tumors. Surgical management was performed and cerebellar tumor was histologically confirmed to be a pilocytic astrocytoma. Spinal tumors were also astrocytomas. The second patient, a 56-year-old woman suffered from right iliac pain, right hemiparesis and motor aphasia. CT revealed two round tumors in the left cerebral hemisphere and bilateral C-P angle tumors. Myelography and MRI demonstrated multiple intradural-extramedullary spinal tumors. Histologically, supratentorial tumors were transitional meningiomas and spinal tumors were neurinomas. It is well known that von Recklinghausen's disease is often associated with brain or spinal tumors. But, in the literature, only 22 cases of von Recklinghausen's disease combined with multiple brain tumors with different histological types and multiple spinal tumors have been reviewed. With our two cases, the average age of these 24 cases was 28.6 years old, nine cases were male and 15 cases were female. All patients had C-P angle tumors and 23 cases were combined with intracranial meningiomas. In this paper, the clinical features and diagnostic aspects were discussed.