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Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease.
J Neurosurg Spine. 2010 Mar; 12(3):233-42.JN

Abstract

OBJECT

Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas.

METHODS

Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed.

RESULTS

One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 +/- 5.0 years (range 0.5-20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54-96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07-55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients).

CONCLUSIONS

Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.

Authors+Show Affiliations

Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.No affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Intramural

Language

eng

PubMed ID

20192620

Citation

Mehta, Gautam U., et al. "Functional Outcome After Resection of Spinal Cord Hemangioblastomas Associated With Von Hippel-Lindau Disease." Journal of Neurosurgery. Spine, vol. 12, no. 3, 2010, pp. 233-42.
Mehta GU, Asthagiri AR, Bakhtian KD, et al. Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. J Neurosurg Spine. 2010;12(3):233-42.
Mehta, G. U., Asthagiri, A. R., Bakhtian, K. D., Auh, S., Oldfield, E. H., & Lonser, R. R. (2010). Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. Journal of Neurosurgery. Spine, 12(3), 233-42. https://doi.org/10.3171/2009.10.SPINE09592
Mehta GU, et al. Functional Outcome After Resection of Spinal Cord Hemangioblastomas Associated With Von Hippel-Lindau Disease. J Neurosurg Spine. 2010;12(3):233-42. PubMed PMID: 20192620.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease. AU - Mehta,Gautam U, AU - Asthagiri,Ashok R, AU - Bakhtian,Kamran D, AU - Auh,Sungyoung, AU - Oldfield,Edward H, AU - Lonser,Russell R, PY - 2010/3/3/entrez PY - 2010/3/3/pubmed PY - 2010/4/7/medline SP - 233 EP - 42 JF - Journal of neurosurgery. Spine JO - J Neurosurg Spine VL - 12 IS - 3 N2 - OBJECT: Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas. METHODS: Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed. RESULTS: One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 +/- 5.0 years (range 0.5-20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54-96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07-55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients). CONCLUSIONS: Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression. SN - 1547-5646 UR - https://www.unboundmedicine.com/medline/citation/20192620/Functional_outcome_after_resection_of_spinal_cord_hemangioblastomas_associated_with_von_Hippel_Lindau_disease_ L2 - https://thejns.org/doi/10.3171/2009.10.SPINE09592 DB - PRIME DP - Unbound Medicine ER -