Top

Published in:

01-09-2006 | Original Paper

Surgical treatment of hemangioblastomas of the central nervous system in pediatric patients

Authors: Vassilios I. Vougioukas, Sven Gläsker, Ulrich Hubbe, Ansgar Berlis, Heymut Omran, Hartmut P. H. Neumann, Vera Van Velthoven

Published in: Child's Nervous System | Issue 9/2006

Abstract

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.

Patients and 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.

Conclusion

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.

Chang SD, Meisel JA, Hancock SL et al (1998) Treatment of hemangioblastomas in von Hippel Lindau disease with linear accelerator based radiosurgery. Neurosurgery 43:28–35CrossRefPubMed

de la Monte SA, Horrowitz SA (1989) Hemangioblastomas: clinical and histopathological factors correlated with recurrence. Neurosurgery 25:695–698CrossRefPubMed

Eskridge JM, McAuliffe W, Harris B (1996) Preoperative endovascular embolisation of craniospinal hemangioblastomas. AJNR Am J Neuroradiol 17:525–531PubMed

Fisher PG, Tontiplaphol A, Pearlman EM et al (2002) Childhood cerebellar hemangioblastoma does not predict germline or somatic mutations in the von Hippel–Lindau disease. Ann Neurol 51:257–260CrossRefPubMed

Glasker S, Bender BU, Apel TW et al (1999) The impact of genetic molecular analysis of the VHL gene in patients with hemangioblastomas of the central nervous system. J Neurol Neurosurg Psychiatry 67:758–762PubMed

Hermann HD, Neuss M, Winkler D (1988) Intramedullary spinal cord tumors resected with CO₂ laser microsurgical technique: recent experience in fifteen patients. Neurosurgery 22:518–522PubMedCrossRef

Latif F, Tory K, Gnarra J et al (1993) Identification of the von Hippel–Lindau disease tumor suppressor gene. Science 260:1317–1320PubMedCrossRef

Lonser RR, Glenn GM, Walther M et al (2003) von Hippel–Lindau disease. Lancet 361:2059–2067CrossRefPubMed

Lonser RR, Weil RJ, Wanebo JE (2003) Surgical management of spinal cord hemangioblastomas in patients with von Hippel–Lindau disease. J Neurosurg 98:106–116PubMed

10.

Maher ER, Yates JRW, Ferguson-Smith MA (1990) Statistical analysis of the two stage mutation model in von Hippel–Lindau disease, and in sporadic cerebellar hemangioblastoma and renal cell carcinoma. J Med Genet 27:311–314PubMed

11.

Neumann HP, Eggert HR, Scheremet R et al (1992) Central nervous system lesions in von Hippel–Lindau syndrome. J Neurol Neurosurg Psychiatry 55:898–901PubMedCrossRef

12.

Neumann HP, Eggert HR, Weigel K et al (1989) Hemangioblastomas of the central nervous system: a 10-year study with special reference to the von Hippel–Lindau syndrome. J Neurosurg 70:24–30PubMed

13.

Roonprapunt C, Silvera VM, Setton A et al (2001) Surgical management of isolated hemangioblastomas of the spinal cord. Neurosurgery 49:321–328CrossRefPubMed

14.

Ries LAG, Eisner MP, Kosary CL et al (2001) SEER cancer statistics review, 1973–1998. National Cancer Institute, Bethesda, MD

15.

Van Velthoven V, Reinacher PC, Klisch J et al (2003) Treatment of intramedullary hemangioblastomas, with special attention to von Hippel–Lindau disease. Neurosurgery 53:1306–1314CrossRefPubMed

16.

Weil RJ, Lonser RR, DeVroom HL et al (2003) Surgical management of brainstem hemangioblastomas in patients with von Hippel–Lindau disease. J Neurosurg 98:95–105PubMed

17.

Wanebo JE, Lonser RR, Glenn GM et al (2003) The natural history of hemangioblastomas of the central nervous system in patients with von Hippel–Lindau disease. J Neurosurg 98:82–94PubMedCrossRef

Title: Surgical treatment of hemangioblastomas of the central nervous system in pediatric patients
Authors: Vassilios I. Vougioukas
Sven Gläsker
Ulrich Hubbe
Ansgar Berlis
Heymut Omran
Hartmut P. H. Neumann
Vera Van Velthoven
Publication date: 01-09-2006
Publisher: Springer-Verlag
Published in: Child's Nervous System / Issue 9/2006
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-005-0018-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Surgical treatment of hemangioblastomas of the central nervous system in pediatric patients

Abstract

Objective

Patients and methods

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Objective

Patients and methods

Results

Conclusion

Please log in to get access to this content

Other articles of this Issue 9/2006

Supratentorial cystic hemangioblastoma with infratentorial extension—a unique location and a rare infant case

Diffuse and focal brain stem tumors in childhood: prognostic factors and surgical outcome

Expansile cranioplasty for massive occipital encephalocele

Spinal arteriovenous shunts presenting before 2 years of age: analysis of 13 cases

Brain surface reformatted imaging (BSRI) in surgical planning for resections around eloquent cortex

Intracranial sinonasal undifferentiated carcinoma (SNUC) in a child