Tags

Type your tag names separated by a space and hit enter

Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease.
J Neurosurg. 2011 May; 114(5):1312-8.JN

Abstract

OBJECT

Brainstem hemangioblastomas are frequently encountered in patients with von Hippel-Lindau (VHL) disease. These tumors can cause significant morbidity, and their optimal management has not been defined. To better define the outcome and management of these tumors, the authors analyzed the long-term results in patients who underwent resection of brainstem hemangioblastomas.

METHODS

Consecutive patients with VHL disease who underwent resection of brainstem hemangioblastomas with a follow-up of 12 months or more were included in this study. Serial functional assessments, radiographic examinations, and operative records were analyzed.

RESULTS

Forty-four patients (17 male and 27 female) underwent 51 operations for resection of 71 brainstem hemangioblastomas. The most common presenting symptoms were headache, swallowing difficulties, singultus, gait difficulties, and sensory abnormalities. The mean follow-up was 5.9 ± 5.0 years (range 1.0-20.8 years). Immediately after 34 operations (66.7%), the patients remained at their preoperative functional status; they improved after 8 operations (15.7%) and worsened after 9 operations (17.6%) as measured by the McCormick scale. Eight (88.9%) of the 9 patients who were worse immediately after resection returned to their preoperative status within 6 months. Two patients experienced functional decline during long-term follow-up (beginning at 2.5 and 5 years postoperatively) caused by extensive VHL disease-associated CNS disease.

CONCLUSIONS

Generally, resection of symptomatic brainstem hemangioblastomas is a safe and effective management strategy in patients with VHL disease. Most patients maintain their preoperative functional status, although long-term decline in functional status may occur due to VHL disease-associated 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 availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

20932100

Citation

Wind, Joshua J., et al. "Long-term Outcome After Resection of Brainstem Hemangioblastomas in Von Hippel-Lindau Disease." Journal of Neurosurgery, vol. 114, no. 5, 2011, pp. 1312-8.
Wind JJ, Bakhtian KD, Sweet JA, et al. Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease. J Neurosurg. 2011;114(5):1312-8.
Wind, J. J., Bakhtian, K. D., Sweet, J. A., Mehta, G. U., Thawani, J. P., Asthagiri, A. R., Oldfield, E. H., & Lonser, R. R. (2011). Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease. Journal of Neurosurgery, 114(5), 1312-8. https://doi.org/10.3171/2010.9.JNS10839
Wind JJ, et al. Long-term Outcome After Resection of Brainstem Hemangioblastomas in Von Hippel-Lindau Disease. J Neurosurg. 2011;114(5):1312-8. PubMed PMID: 20932100.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Long-term outcome after resection of brainstem hemangioblastomas in von Hippel-Lindau disease. AU - Wind,Joshua J, AU - Bakhtian,Kamran D, AU - Sweet,Jennifer A, AU - Mehta,Gautam U, AU - Thawani,Jayesh P, AU - Asthagiri,Ashok R, AU - Oldfield,Edward H, AU - Lonser,Russell R, Y1 - 2010/10/08/ PY - 2010/10/12/entrez PY - 2010/10/12/pubmed PY - 2011/7/1/medline SP - 1312 EP - 8 JF - Journal of neurosurgery JO - J Neurosurg VL - 114 IS - 5 N2 - OBJECT: Brainstem hemangioblastomas are frequently encountered in patients with von Hippel-Lindau (VHL) disease. These tumors can cause significant morbidity, and their optimal management has not been defined. To better define the outcome and management of these tumors, the authors analyzed the long-term results in patients who underwent resection of brainstem hemangioblastomas. METHODS: Consecutive patients with VHL disease who underwent resection of brainstem hemangioblastomas with a follow-up of 12 months or more were included in this study. Serial functional assessments, radiographic examinations, and operative records were analyzed. RESULTS: Forty-four patients (17 male and 27 female) underwent 51 operations for resection of 71 brainstem hemangioblastomas. The most common presenting symptoms were headache, swallowing difficulties, singultus, gait difficulties, and sensory abnormalities. The mean follow-up was 5.9 ± 5.0 years (range 1.0-20.8 years). Immediately after 34 operations (66.7%), the patients remained at their preoperative functional status; they improved after 8 operations (15.7%) and worsened after 9 operations (17.6%) as measured by the McCormick scale. Eight (88.9%) of the 9 patients who were worse immediately after resection returned to their preoperative status within 6 months. Two patients experienced functional decline during long-term follow-up (beginning at 2.5 and 5 years postoperatively) caused by extensive VHL disease-associated CNS disease. CONCLUSIONS: Generally, resection of symptomatic brainstem hemangioblastomas is a safe and effective management strategy in patients with VHL disease. Most patients maintain their preoperative functional status, although long-term decline in functional status may occur due to VHL disease-associated progression. SN - 1933-0693 UR - https://www.unboundmedicine.com/medline/citation/20932100/Long_term_outcome_after_resection_of_brainstem_hemangioblastomas_in_von_Hippel_Lindau_disease_ L2 - https://thejns.org/doi/10.3171/2010.9.JNS10839 DB - PRIME DP - Unbound Medicine ER -