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Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease.
J Neurosurg. 2003 Jan; 98(1):95-105.JN

Abstract

OBJECT

Hemangioblastomas of the brainstem constitute 5 to 10% of central nervous system (CNS) tumors in patients with von Hippel-Lindau (VHL) disease. At present, optimal management of brainstem hemangioblastomas associated with VHL disease is incompletely defined. In an attempt to clarify some of the uncertainty about the operative treatment of these lesions and its outcome, the authors reviewed all cases of VHL disease in which resection of brainstem hemangioblastomas was performed at the National Institutes of Health during a 10-year period.

METHODS

Twelve consecutive patients with VHL disease (six male and six female patients [mean age 31.7 +/- 9 years; range 15-46 years]) who underwent 13 operations to remove 17 brainstem hemangioblastomas were included in this study (mean follow-up period, 88.4 +/- 37.4 months; range 37-144 months). Serial examinations, hospital charts, magnetic resonance images, and operative records were reviewed. To evaluate clinical course, clinical grades were assigned to each patient before and after surgery. Preoperative neurological function was the best predictor of long-term outcome. In addition, patients who underwent CNS surgeries for hemangioblastomas were more likely to improve or to remain neurologically stable. Tumor or cyst size, the presence of a cyst, or the location of the tumor (intramedullary, extramedullary, or mixed; posterior medullary, obex, or lateral) did not affect outcome. No patient was neurologically worse after brainstem surgery. At long-term follow-up review (mean 88.4 months), only one patient had declined neurologically and this was due to the cumulative neurological effects caused by eight additional hemangioblastomas of the spinal cord and their surgical treatment.

CONCLUSIONS

Brainstem hemangioblastomas in patients with VHL disease can be removed safely; they generally should be resected when they become symptomatic or when the tumor has reached a size such that further growth will increase the risks associated with surgery, or in the presence of an enlarging cyst. Magnetic resonance imaging is usually sufficient for preoperative evaluation and presurgical embolization is unnecessary. The goal of surgery is complete resection of the lesion before the patient experiences a disabling neurological deficit.

Authors+Show Affiliations

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA. weilr@ninds.nih.govNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

12546357

Citation

Weil, Robert J., et al. "Surgical Management of Brainstem Hemangioblastomas in Patients With Von Hippel-Lindau Disease." Journal of Neurosurgery, vol. 98, no. 1, 2003, pp. 95-105.
Weil RJ, Lonser RR, DeVroom HL, et al. Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. J Neurosurg. 2003;98(1):95-105.
Weil, R. J., Lonser, R. R., DeVroom, H. L., Wanebo, J. E., & Oldfield, E. H. (2003). Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. Journal of Neurosurgery, 98(1), 95-105.
Weil RJ, et al. Surgical Management of Brainstem Hemangioblastomas in Patients With Von Hippel-Lindau Disease. J Neurosurg. 2003;98(1):95-105. PubMed PMID: 12546357.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Surgical management of brainstem hemangioblastomas in patients with von Hippel-Lindau disease. AU - Weil,Robert J, AU - Lonser,Russell R, AU - DeVroom,Hetty L, AU - Wanebo,John E, AU - Oldfield,Edward H, PY - 2003/1/28/pubmed PY - 2003/2/14/medline PY - 2003/1/28/entrez SP - 95 EP - 105 JF - Journal of neurosurgery JO - J Neurosurg VL - 98 IS - 1 N2 - OBJECT: Hemangioblastomas of the brainstem constitute 5 to 10% of central nervous system (CNS) tumors in patients with von Hippel-Lindau (VHL) disease. At present, optimal management of brainstem hemangioblastomas associated with VHL disease is incompletely defined. In an attempt to clarify some of the uncertainty about the operative treatment of these lesions and its outcome, the authors reviewed all cases of VHL disease in which resection of brainstem hemangioblastomas was performed at the National Institutes of Health during a 10-year period. METHODS: Twelve consecutive patients with VHL disease (six male and six female patients [mean age 31.7 +/- 9 years; range 15-46 years]) who underwent 13 operations to remove 17 brainstem hemangioblastomas were included in this study (mean follow-up period, 88.4 +/- 37.4 months; range 37-144 months). Serial examinations, hospital charts, magnetic resonance images, and operative records were reviewed. To evaluate clinical course, clinical grades were assigned to each patient before and after surgery. Preoperative neurological function was the best predictor of long-term outcome. In addition, patients who underwent CNS surgeries for hemangioblastomas were more likely to improve or to remain neurologically stable. Tumor or cyst size, the presence of a cyst, or the location of the tumor (intramedullary, extramedullary, or mixed; posterior medullary, obex, or lateral) did not affect outcome. No patient was neurologically worse after brainstem surgery. At long-term follow-up review (mean 88.4 months), only one patient had declined neurologically and this was due to the cumulative neurological effects caused by eight additional hemangioblastomas of the spinal cord and their surgical treatment. CONCLUSIONS: Brainstem hemangioblastomas in patients with VHL disease can be removed safely; they generally should be resected when they become symptomatic or when the tumor has reached a size such that further growth will increase the risks associated with surgery, or in the presence of an enlarging cyst. Magnetic resonance imaging is usually sufficient for preoperative evaluation and presurgical embolization is unnecessary. The goal of surgery is complete resection of the lesion before the patient experiences a disabling neurological deficit. SN - 0022-3085 UR - https://www.unboundmedicine.com/medline/citation/12546357/Surgical_management_of_brainstem_hemangioblastomas_in_patients_with_von_Hippel_Lindau_disease_ L2 - https://thejns.org/doi/10.3171/jns.2003.98.1.0095 DB - PRIME DP - Unbound Medicine ER -