Sacral Hemangioblastoma in a Patient With von Hippel-Lindau Disease

Ryszard M. Pluta, M.D., Scott D. Wait, B.A., John A. Butman, M.D., Kathleen A. Leppig, M.D., Alexander O. Vortmeyer, M.D., Edward H. Oldfield, M.D., Russell R. Lonser, M.D.


Neurosurg Focus. 2003;15(2) 

In This Article

Abstract and Introduction

Hemangioblastomas are histologically benign neoplasms that occur sporadically or as part of von Hippel-Lindau disease. Hemangioblastomas may occur anywhere along the neuraxis, but sacral hemangioblastomas are extremely rare. To identify features that will help guide the operative and clinical management of these lesions, the authors describe the management of a large von Hippel-Lindau disease associated sacral hemangioblastoma and review the literature.

The authors present the case of a 38-year-old woman with von Hippel-Lindau disease and a 10-year history of progressive back pain, as well as left lower-extremity pain and numbness. Neurological examination revealed decreased sensation in the left S-1 and S-2 dermatomes. Magnetic resonance imaging demonstrated a large enhancing lesion in the sacral region, with associated erosion of the sacrum. The patient underwent arteriography and embolization of the tumor and then resection. The histopathological diagnosis was consistent with hemangioblastoma and showed intrafascicular tumor infiltration of the S-2 nerve root. At 1-year follow-up examination, pain had resolved and numbness improved.

Sacral nerve root hemangioblastomas may be safely removed in most patients, resulting in stabilization or improvement in symptomatology. Generally, hemangioblastomas of the sacral nerve roots should be removed when they cause symptoms. Because they originate from the nerve root, the nerve root from which the hemangioblastoma originates must be sacrificed to achieve complete resection.

Hemangioblastomas are histologically benign, highly vascular tumors that develop spontaneously or as part of von Hippel-Lindau disease. Hemangioblastomas (sporadic or associated with von Hippel-Lindau disease) can be found throughout the CNS, but sacral hemangioblastomas are extremely rare.[12,23] Thus, little information exists regarding their prevalence, clinical manifestation, and management. Sporadic and von Hippel-Lindau disease associated hemangioblastomas are histologically identical, and despite being classified as benign neoplasms they can cause significant neurological deficits related to their location and size.[3] Whether they occur sporadically or in relationship to von Hippel-Lindau disease, they can be cured by complete resection.[12,13]