Introduction: Hemangioblastomas are benign tumors, present at Central Nervous System in at least 75% of patients affected from von Hippel-Lindau ( VHL) disease. Their spinal location on spinal roots, at intradural level, is highly rare. The target of the present paper is to review the clinical and surgical treatment aspects in a series of VHL cases.
Methods: A retrospective review of clinical and surgical reports has been performed on patients treated for spinal root hemangioblastomas within a series of 71 VHL patients, followed and treated in a neurosurgery unit dedicated to Familial Neurooncology patients. A review on published spinal root hemangioblastomas has been performed, comparing the results in order to get a common pattern of behavior and management .
Results: In our series, a total of five surgical procedures in three patients have been performed, to treat spinal root hemangioblastomas. Age of presentation was between 41 and 55. The most frequent symptoms were spinal sensitive, and motor deficit, both in two. The anatomical level was cervical spine in 1 case, lumbar in 2, and sacral in 2 more cases. On neurophysiological intraoperative tumor dissection monitoring, sensitive and no motor deficit was observed. In fact, in our cases, tumor was related to sensitive root in all (5/5) of hemangioblastomas. In postoperative follow-up, a clinical maintenance level was observed in pre-and postoperative, evaluation following Mc´Cormicks performance scale. Hystopathological evaluation showed neurofilament fibers encased within tumor tissue, in most of cases.
Conclusions: Up to now, 31 spinal root hemangioblastomas have been previously published. We described five additional cases. Tumors grow more frequently on sensitive spinal root fibers, making possible a total resection of hemangioblastomas with minimal or no functional deficits in VHL patients. We suggest that surgical treatment on spinal root hemangioblastomas be performed at clinical symptom beginning, in order to get the best treatment results.
Patient Care: In von Hippel-Lindau patients, a rare disease with frequent spinal tumors expression, knowledge of the behavior, their particular relation with the sensitive spinal root sparing the motor roots, and the management of these spinal root hemangioblastomas will improve the outcome of treatment of VHL patients.
Learning Objectives: Management of spinal hemangioblastomas in von Hippel-Lindau patients, particularly hemangioblastomas presented as spinal root tumors. Most of tumors are related to sensitive roots, and can be resected without motor deficits if carefull microsugical dissection is performed when resection.