SPINAL LEPTOMENINGEAL HEMANGIOBLASTOMATOSIS IN VON-HIPPEL-LINDAU-DISEASE - MAGNETIC-RESONANCE AND PATHOLOGICAL FINDINGS

A 55-year-old man with von Hippel-Lindau disease presented with quadri paresis. Multiple enhancing cervical and thoracic spinal masses were s een on magnetic resonance imaging (MRI). A rim of diffuse, nodular enh ancement linking all of the discrete masses was apparent on the surfac e of the cervical and thoracic regions of the cord. Surgical explorati on revealed multiple extramedullary-intradural and intramedullary mass es, extending to and infiltrating the cord; the leptomeninges containe d numerous small tumor seeds at several levels. The excised spinal mas ses were diagnosed as capillary hemangioblastomas, which infiltrated t he pia mater. Diffuse, intense, spinal leptomeningeal enhancement on M RI associated with multiple hemangioblastomas has not been previously reported and may be referred to as spinal ''leptomeningeal hemangiobla stomatosis.''