Abstract
Purpose Primary optic disc tumors are often a challenge for ophthalmologists. They have very
different appearances, and many primary optic disc tumors are associated with syndromic
diseases (especially phakomatoses). Because of the rarity of primary optic disc
tumors, classification and assessment are often difficult.
Material and Methods A systematic search in the electronic patient files (period 01.01.2015 – 01.06.2022)
of the Department of Ophthalmology of the University of Münster Medical
Center for patients with primary optic disc tumors was performed. For each tumor
entity, exemplary cases were selected, which are presented here in detail. The criteria
for the exemplary
case selection were a clear diagnosis, the presence of suitable image material
and follow-up examinations in our clinic.
Results The search yielded seven cases with three different primary tumor entities in the
optic disc region (capillary hemangioblastoma, astrocytic hamartoma and melanocytoma).
Four
patients were selected as examples and are presented here: two cases for capillary
hemangioblastoma (one isolated and the other in the context of Von-Hippel-Lindau syndrome)
and one case
each for astrocytic hamartoma and melanocytoma). We outline the further diagnosis
and the course of the disease and we give an overview of the essential features of
the underlying tumors in
each case.
Conclusion The knowledge of the different primary tumors of the optic disc is necessary for
a correct diagnosis and for the differentiation from malignant processes and optic
disc
anomalies. In many cases, further interdisciplinary diagnostics are necessary.
Multimodal imaging is helpful and a referral to a center for ocular tumors is worth
considering.
Key words
optic disc - tumor - capillary hemangioblastoma - astrocytic hamartoma - melanocytoma
- optic nerve
