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 quadriparesis. Multiple enhancing cervical and thoracic spinal masses were seen on magnetic resonance imaging (MRI). A rim of diffuse, nodular enhancement linking all of the discrete masses was apparent on the surface of the cervical and thoracic regions of the cord. Surgical exploration revealed multiple extramedullary-intradural and intramedullary masses, extending to and infiltrating the cord; the leptomeninges contained numerous small tumor seeds at several levels. The excised spinal masses were diagnosed as capillary hemangioblastomas, which infiltrated the pia mater. Diffuse, intense, spinal leptomeningeal enhancement on MRI associated with multiple hemangioblastomas has not been previously reported and may be referred to as spinal 'leptomeningeal hemangioblastomatosis'.