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Diagnosis and treatment strategy of hemangioblastomas of the optic nerve and chiasm
Author(s): ,
E. Vergauwen
Affiliations:
Universitair Ziekenhuis Brussel, Neurosurgery, Jette, Belgium
,
M.T. Krüger
Affiliations:
Freiburg University Medical Center, Neurosurgery, Freiburg, Germany
,
J.-H. Klingler
Affiliations:
Freiburg University Medical Center, Neurosurgery, Freiburg, Germany
,
V. Van Velthoven
Affiliations:
Universitair Ziekenhuis Brussel, Neurosurgery, Jette, Belgium
S. Gläsker
Affiliations:
Universitair Ziekenhuis Brussel, Neurosurgery, Jette, Belgium
EANS Academy. Vergauwen E. Oct 21, 2018; 225846; EP3079
Dr. Evelynn Vergauwen
Dr. Evelynn Vergauwen
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Abstract
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Objective: Optic nerve and chiasm hemangioblastomas are rare tumors, occurring sporadically or in the context of von Hippel-Lindau (VHL) disease. They have only been portrayed in isolated case reports and small cohorts. Their natural history and therapeutic strategies are therefore not well described. To better characterize these rare tumors, we retrospectively analyzed our hemangioblastoma patient series. By combining our own experience to a review of all known cases in literature, we intend to create the first treatment standard for optic nerve and chiasm hemangioblastomas.

Methods: We reviewed 2 electronic databases in the hospitals of our senior authors, searching for VHL patients with optic nerve or chiasm hemangioblastomas. Clinical data were summarized. Tumor size and growth rate were measured on contrast enhanced MRI and correlated to visual symptoms. If available, comparable data were collected from cases already described in literature.

Results: Ten of 269 VHL patients had optic nerve or chiasm hemangioblastomas. In 9 of 10 patients, tumors were diagnosed incidentally upon annual MRI screening. Subsequent radiological follow-up periods ranged from 3 months to 11 years. Seven of these 9 asymptomatic patients had absent or very slow annual progression, without developing visual deficits. Two of these initially asymptomatic patients suffered from rapid tumor growth and progressive visual deficits. Both underwent late stage surgery, resulting in incomplete resection and progressive visual deficits. One VHL patient already had partial visual loss at time of diagnosis. A watchful-waiting approach was preferred because the hemangioblastoma was ineligible for vision-sparing surgery.

Conclusion: When optic nerve and chiasm hemangioblastomas are diagnosed, annual MRI follow-up is sufficient, as long as patients do not develop visual deficits. If tumors grow fast and/or patients develop visual deficits, surgical resection must be considered because neurological deficits are irreversible and resection of large tumors carries a higher risk of further visual decline.
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