gms | German Medical Science

Objective: Hemangioblastomas of the optic nerve and chiasm have only been described in single cases and small case series. The natural history of these tumors, therapeutic strategies and the surgical indications are therefore not well characterized. To clarify therapeutic strategies for these rare tumors we performed a retrospective study on one of the largest series of VHL patients published so far.

Method: The clinical course, magnetic resonance imaging findings and histological features of nine patients with VHL and hemangioblastomas affecting the optic nerve and chiasm were reviewed.

Results: In 9 patients hemangioblastomas of the optic nerve or chiasm were diagnosed by MRI. The follow-up period started at the time of diagnosis and ranged from 3 months to 11 years with a mean of 3,8 years. 7 patients showed no or only slow progress in annual MRI scans. None of these patients developed visual deficits. Two patients showed rapid tumor growth and both developed progressive visual deficits. Both underwent surgery at a late stage when tumors had grown big resulting in an incomplete resection and progressive visual deficits.

Conclusions: If hemangioblastomas of the optic nerve and chiasm are diagnosed an annual routine follow-up is sufficient, as long as patients do not develop visual deficits. If tumors grow fast and/or patients develop visual deficits we recommend a resection because neurological deficits are not reversible and the resection of large tumors carries a higher risk of further decline of visual functions.