Özet:

Radiological examinations of a 21-year-old man with headache and gait abnormality revealed hemangioblastomas in both cerebellar hemispheres and in the cervical spinal cord. There were also other findings consistent with the diagnosis of von Hippel-Lindau syndrome (VHLS), but no retinal hemangioblastomas were detected. The patient underwent bilateral suboccipital craniectomy for removal of the cerebellar tumors. Three months after this surgery, a C6 laminectomy was performed and the cervical hemangioblastoma was also totally excised. In the imrnediate postoperative period, the patient developed motor weakness and radiating pain in his right leg. Physical examination and a computerized tomography sean revealed a well-circumscribed solid mass in his right posterior thigh, which was thought to be sciatic nerve tumor. A third operation was done to totally excise this mass. Histological examination identified the neoplasm as a schwannoma. Although we considered the presence of a sciatic nerve schwannoma in this patient to be coincidental, we believe that maintaining a high index of suspicion for clinically silent peripheral nerve sheath tumors in individuals with VHLS may reveal that these masses are yet another manifestation of this syndrome. Already more than 30 lesions characteristic of this condition have been described to date. This is the first reported case of VHLS accompanied by a peripheral nerve sheath tumor.

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