A Case of Holocord Leptomeningeal Dissemination from Cerebellar Hemangioblastoma without von Hippel-Lindau Disease.

Abstract

Hemangioblastoma disseminated along leptomeninges from the solitary cranial lesion without von Hippel-Lindau (VHL) disease is a quite rare instance with 23 cases reported in 40 years. We add a new case and discuss these rare instances. A 55-year-old female underwent surgery for total removal of cerebellar hemangioblastoma. Twenty months later, magnetic resonance (MR) images of the spinal cord revealed a tumor compressing the thoracic cord at T3-4 level which was removed en bloc by emergent spinal surgery. However, paraplegia and bowel bladder dysfunction recurred 5 months after the spinal surgery. Spine MR images showed diffuse enhancement of subarachnoid space. Exploratory surgery disclosed that the enhanced lesion was disseminated hemangioblastoma. After whole spinal irradiation, she was transferred to a palliative care hospital. Even after complete removal, possibility of leptomeningeal dissemination demands continuous follow-up. The mechanism of seeding of hemangioblastoma remains unclear, but attention must be paid to avoid spreading tumor cells during surgery because all the disseminated cases had precedent cranial surgery.