Retrobulbar Hemangioblastomas in von Hippel-Lindau Disease: Clinical Course and Management.
Optic nerve hemangioblastoma
Retrobulbar hemangioblastoma
von Hippel-Lindau
Journal
Neurosurgery
ISSN: 1524-4040
Titre abrégé: Neurosurgery
Pays: United States
ID NLM: 7802914
Informations de publication
Date de publication:
15 04 2021
15 04 2021
Historique:
received:
12
08
2020
accepted:
04
11
2020
pubmed:
15
1
2021
medline:
3
7
2021
entrez:
14
1
2021
Statut:
ppublish
Résumé
Retrobulbar hemangioblastomas involving the optic apparatus in patients with von Hippel-Lindau disease (VHL) are rare, with only 25 reported cases in the literature. To analyze the natural history of retrobulbar hemangioblastomas in a large cohort of VHL patients in order to define presentation, progression, and management. Clinical history and imaging of 250 patients with VHL in an ongoing natural history trial and 1774 patients in a neurosurgical protocol were reviewed. The clinical course, magnetic resonance images, treatment, and outcomes were reviewed for all included patients. A total of 18 patients with retrobulbar hemangioblastoma on surveillance magnetic resonance imaging met the inclusion criteria for this study. Of the 17 for whom clinical information was available, 10 patients presented with symptoms related to the hemangioblastoma, and 7 were asymptomatic. The mean tumor volume was larger for symptomatic (810.6 ± 545.5 mm3) compared to asymptomatic patients (307.6 ± 245.5 mm3; P < .05). A total of 5 of the symptomatic patients were treated surgically and all experienced improvement in their symptoms. All 3 symptomatic patients that did not undergo intervention had continued symptom progression. Long-term serial imaging on asymptomatic patients showed that these tumors can remain radiographically stable and asymptomatic for extended periods of time (101.43 ± 71 mo). This study suggests that retrobulbar hemangioblastomas may remain stable and clinically asymptomatic for long durations. Recent growth and larger tumor volume were associated with symptom occurrence. Surgical treatment of symptomatic retrobulbar hemangioblastomas can be safe and may reverse the associated symptoms.
Sections du résumé
BACKGROUND
Retrobulbar hemangioblastomas involving the optic apparatus in patients with von Hippel-Lindau disease (VHL) are rare, with only 25 reported cases in the literature.
OBJECTIVE
To analyze the natural history of retrobulbar hemangioblastomas in a large cohort of VHL patients in order to define presentation, progression, and management.
METHODS
Clinical history and imaging of 250 patients with VHL in an ongoing natural history trial and 1774 patients in a neurosurgical protocol were reviewed. The clinical course, magnetic resonance images, treatment, and outcomes were reviewed for all included patients.
RESULTS
A total of 18 patients with retrobulbar hemangioblastoma on surveillance magnetic resonance imaging met the inclusion criteria for this study. Of the 17 for whom clinical information was available, 10 patients presented with symptoms related to the hemangioblastoma, and 7 were asymptomatic. The mean tumor volume was larger for symptomatic (810.6 ± 545.5 mm3) compared to asymptomatic patients (307.6 ± 245.5 mm3; P < .05). A total of 5 of the symptomatic patients were treated surgically and all experienced improvement in their symptoms. All 3 symptomatic patients that did not undergo intervention had continued symptom progression. Long-term serial imaging on asymptomatic patients showed that these tumors can remain radiographically stable and asymptomatic for extended periods of time (101.43 ± 71 mo).
CONCLUSION
This study suggests that retrobulbar hemangioblastomas may remain stable and clinically asymptomatic for long durations. Recent growth and larger tumor volume were associated with symptom occurrence. Surgical treatment of symptomatic retrobulbar hemangioblastomas can be safe and may reverse the associated symptoms.
Identifiants
pubmed: 33442737
pii: 6096353
doi: 10.1093/neuros/nyaa565
pmc: PMC8223245
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, N.I.H., Intramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
1012-1020Informations de copyright
Published by Oxford University Press on behalf of Congress of Neurological Surgeons 2021.
Références
Neurosurgery. 1995 Mar;36(3):573-80; discussion 580-1
pubmed: 7753357
J Neurosurg. 2009 Feb;110(2):350-3
pubmed: 18834262
Turk Neurosurg. 2017;27(5):827-831
pubmed: 27509455
Ophthalmology. 1988 Mar;95(3):398-402
pubmed: 3050687
World Neurosurg. 2019 Aug;128:211-215
pubmed: 31054346
Acta Neurochir Suppl. 2005;94:17-21
pubmed: 16060236
Ophthalmology. 2002 Oct;109(10):1799-806
pubmed: 12359597
Retina. 2019 Dec;39(12):2254-2263
pubmed: 31259811
Ophthalmol Retina. 2017 Jan - Feb;1(1):59-67
pubmed: 31047395
Surg Neurol Int. 2014 Mar 06;5:33
pubmed: 24778921
Ophthalmology. 2008 Aug;115(8):1382-9
pubmed: 18395800
Arch Soc Esp Oftalmol. 2006 May;81(5):293-6
pubmed: 16752322
Neurology. 1991 Jan;41(1):41-6
pubmed: 1985294
J Neurosurg. 1989 Jan;70(1):24-30
pubmed: 2909683
Lancet. 2003 Jun 14;361(9374):2059-67
pubmed: 12814730
J Neurosurg. 2003 Jan;98(1):82-94
pubmed: 12546356
Neuroophthalmology. 2014 Sep 19;38(5):254-256
pubmed: 27928308
Science. 1993 May 28;260(5112):1317-20
pubmed: 8493574
Br J Neurosurg. 2009;23(5):561-3
pubmed: 19718547
JAMA Neurol. 2018 May 1;75(5):620-627
pubmed: 29379961
Sci Rep. 2017 Jan 17;7:40822
pubmed: 28094316
J Neurosurg. 2008 Aug;109(2):313-7
pubmed: 18671645
JAMA. 2008 Sep 17;300(11):1334-42
pubmed: 18799446
Ophthalmology. 2008 Nov;115(11):2095
pubmed: 19068378
Ophthalmology. 2002 Sep;109(9):1745-51
pubmed: 12208726
J Neurosurg. 2000 Jun;92(6):1028-35
pubmed: 10839266
AJR Am J Roentgenol. 1992 Aug;159(2):403-5
pubmed: 1632366
Neurosurgery. 2010 Sep;67(3):577-87; discussion 587
pubmed: 20647972
Ocul Oncol Pathol. 2018 Nov;4(6):370-374
pubmed: 30574489
J Neurol Neurosurg Psychiatry. 1990 Oct;53(10):890-5
pubmed: 2266371
J Neuroimaging. 1997 Jan;7(1):48-50
pubmed: 9038433
J Kidney Cancer VHL. 2018 Jun 06;5(2):1-6
pubmed: 29911000
Surv Ophthalmol. 1995 Jan-Feb;39(4):302-6
pubmed: 7725229