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Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study.
Oper Neurosurg (Hagerstown). 2017 08 01; 13(4):435-440.ON

Abstract

BACKGROUND

Cauda equina hemangioblastomas in von Hippel-Lindau (VHL) disease can cause significant neurological signs and symptoms. Despite their associated morbidity, the management of these tumors remains incompletely defined.

OBJECTIVE

To determine optimal management, we analyzed the functional outcomes after resection of these tumors.

METHODS

VHL patients who underwent surgical resection of cauda equina hemangioblastomas at the National Institutes of Health and the University of Virginia were included. Clinical and radiological follow-up was performed at 6- to 12-month intervals after surgery.

RESULTS

Fifteen patients underwent 18 operations for 21 cauda equina hemangioblastomas (median follow-up 5.9 years). Patients often presented with multiple symptoms, including pain (67%), numbness (50%), urinary complaints (33%), and weakness (11%). Median preoperative tumor volume was 1.2 cm 3 . Four tumors at 3 operations were not resected due to a motor nerve root origin. Gross total resection was achieved in 14 surgeries (93% of operations when resection was attempted). New mild (non-function limiting) neurological symptoms were noted after 11 operations (61%), which most often (64%) resolved within 2 weeks of surgery. At 6-month follow-up, 15 patients (83%) were stable, 2 (11%) were improved, and 1 (6%) was worse. Histological analysis revealed that all tumors originated from within the involved nerve fascicle.

CONCLUSIONS

VHL-associated cauda equina hemangioblastomas have an intrafascicular origin and require interruption of the rootlet of origin for complete resection. Motor nerve root involvement may preclude complete resection but strategies including bony decompression and/or interruption of vascular supply may provide a therapeutic option. Nevertheless, most VHL patients with symptom-producing lesions improve with resection.

Authors+Show Affiliations

Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, University of Virginia Health Sciences Center, University of Virginia, Charlottesville, Virginia.Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, University of Virginia Health Sciences Center, University of Virginia, Charlottesville, Virginia.Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland.Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, University of Virginia Health Sciences Center, University of Virginia, Charlottesville, Virginia.Surgical Neurology Branch, National Institutes of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland. Department of Neurosurgery, Ohio State University Wexner Medical Center, Columbus, Ohio.

Pub Type(s)

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

Language

eng

PubMed ID

28838111

Citation

Mehta, Gautam U., et al. "Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: an Observational Cohort Study." Operative Neurosurgery (Hagerstown, Md.), vol. 13, no. 4, 2017, pp. 435-440.
Mehta GU, Montgomery BK, Maggio DM, et al. Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study. Oper Neurosurg (Hagerstown). 2017;13(4):435-440.
Mehta, G. U., Montgomery, B. K., Maggio, D. M., Chittiboina, P., Oldfield, E. H., & Lonser, R. R. (2017). Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study. Operative Neurosurgery (Hagerstown, Md.), 13(4), 435-440. https://doi.org/10.1093/ons/opx019
Mehta GU, et al. Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: an Observational Cohort Study. Oper Neurosurg (Hagerstown). 2017 08 1;13(4):435-440. PubMed PMID: 28838111.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Functional Outcome After Resection of Von Hippel-Lindau Disease-Associated Cauda Equina Hemangioblastomas: An Observational Cohort Study. AU - Mehta,Gautam U, AU - Montgomery,Blake K, AU - Maggio,Dominic M, AU - Chittiboina,Prashant, AU - Oldfield,Edward H, AU - Lonser,Russell R, PY - 2016/07/24/received PY - 2017/01/28/accepted PY - 2017/8/26/entrez PY - 2017/8/26/pubmed PY - 2019/7/10/medline KW - Cauda equina KW - Hemangioblastoma KW - Lumbosacral KW - Nerve root KW - von Hippel-Lindau disease SP - 435 EP - 440 JF - Operative neurosurgery (Hagerstown, Md.) JO - Oper Neurosurg (Hagerstown) VL - 13 IS - 4 N2 - BACKGROUND: Cauda equina hemangioblastomas in von Hippel-Lindau (VHL) disease can cause significant neurological signs and symptoms. Despite their associated morbidity, the management of these tumors remains incompletely defined. OBJECTIVE: To determine optimal management, we analyzed the functional outcomes after resection of these tumors. METHODS: VHL patients who underwent surgical resection of cauda equina hemangioblastomas at the National Institutes of Health and the University of Virginia were included. Clinical and radiological follow-up was performed at 6- to 12-month intervals after surgery. RESULTS: Fifteen patients underwent 18 operations for 21 cauda equina hemangioblastomas (median follow-up 5.9 years). Patients often presented with multiple symptoms, including pain (67%), numbness (50%), urinary complaints (33%), and weakness (11%). Median preoperative tumor volume was 1.2 cm 3 . Four tumors at 3 operations were not resected due to a motor nerve root origin. Gross total resection was achieved in 14 surgeries (93% of operations when resection was attempted). New mild (non-function limiting) neurological symptoms were noted after 11 operations (61%), which most often (64%) resolved within 2 weeks of surgery. At 6-month follow-up, 15 patients (83%) were stable, 2 (11%) were improved, and 1 (6%) was worse. Histological analysis revealed that all tumors originated from within the involved nerve fascicle. CONCLUSIONS: VHL-associated cauda equina hemangioblastomas have an intrafascicular origin and require interruption of the rootlet of origin for complete resection. Motor nerve root involvement may preclude complete resection but strategies including bony decompression and/or interruption of vascular supply may provide a therapeutic option. Nevertheless, most VHL patients with symptom-producing lesions improve with resection. SN - 2332-4260 UR - https://www.unboundmedicine.com/medline/citation/28838111/Functional_Outcome_After_Resection_of_Von_Hippel_Lindau_Disease_Associated_Cauda_Equina_Hemangioblastomas:_An_Observational_Cohort_Study_ L2 - https://academic.oup.com/ons/article-lookup/doi/10.1093/ons/opx019 DB - PRIME DP - Unbound Medicine ER -