Surgical treatment of hemangioblastomas of the central nervous system in pediatric patients.
OBJECTIVE
Hemangioblastomas are histologically benign lesions that occur sporadically or as a manifestation of von Hippel-Lindau disease (VHL). The treatment strategy of these neoplasms is complicated by their unpredictable growth patterns and the often irreversible neurological deficits they may cause. This study aims to outline the neurosurgical treatment options and to address the ongoing debate of surgical timing in pediatric patients with VHL.


METHODS
Thirteen consecutive pediatric patients (mean age 15.1 years) who were surgically treated for intracranial (n=8) or spinal hemangioblastomas (n=5) were included in this study (range of clinical follow up 12-86 months). Ten patients were affected by von Hippel-Lindau and three were with sporadic tumors. Serial examinations, preoperative MRI studies, and operative findings were reviewed.


RESULTS
Patients with cerebellar and intramedullary hemangioblastomas did not develop additional neurological deficits postoperatively. Two patients with brainstem tumors exhibited transient hemiparesis and caudal nerve palsy, respectively. Both patients recovered completely from their postoperative deficits. Preoperatively symptomatic patients with spinal tumors did not deteriorate nor improve after surgery. During the observed follow-up periods, no tumor recurrences were observed.


CONCLUSIONS
Central nervous system (CNS) hemangioblastomas in pediatric patients can be surgically treated with low morbidity. Based on our experience, we recommend considering also the surgical removal of asymptomatic hemangioblastomas with proven radiological progression to prevent the development of permanent neurological deficits. Molecular screening of every pediatric patient and family is mandatory to enable the detection of extraneurological tumors and the development of an efficient therapeutic strategy.
Vougioukas
Vassilios I
VI
Department of Neurosurgery, Neurocenter, Albert-Ludwigs-University of Freiburg, Breisacherstrasse 64, 79106 Freiburg im Breisgau, Germany. vvougio@nz.ukl.uni-freiburg.de
Gläsker
Sven
S
Hubbe
Ulrich
U
Berlis
Ansgar
A
Omran
Heymut
H
Neumann
Hartmut P H
HP
Van Velthoven
Vera
V
D000293:Adolescent; D001933:Brain Stem / Q000473:pathology / Q000601:surgery; D020295:Brain Stem Neoplasms / Q000175:diagnosis / Q000601:surgery*; D002528:Cerebellar Neoplasms / Q000175:diagnosis / Q000601:surgery*; D002531:Cerebellum / Q000473:pathology / Q000601:surgery; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D018325:Hemangioblastoma / Q000175:diagnosis / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D009460:Neurologic Examination; D011183:Postoperative Complications / Q000175:diagnosis; D013116:Spinal Cord / Q000473:pathology / Q000601:surgery; D013120:Spinal Cord Neoplasms / Q000175:diagnosis / Q000601:surgery*; D006623:von Hippel-Lindau Disease / Q000175:diagnosis / Q000601:surgery*