Lymphoma of the eye and its adnexa : Modern pathological diagnostics and systemic treatment

Abstract

Malignant lymphomas of the eye and its adnexal structures account for approximately 5-15% of extranodal lymphomas. According to anatomic and biological criteria, two large groups of lymphomas in and around the eye need to be distinguished: (1)primary lymphomas of intraocular structures and (2)primary lymphomas of ocular adnexa. Furthermore, there is alarge spectrum of secondary manifestations of malignant lymphomas in ocular and periocular structures. This article gives a summary of the classification and molecular pathology of various intraocular and periocular lymphomas as well as oncological systemic treatment with a focus on primary vitreoretinal lymphomas. A selective literature search was carried out in PubMed on the topic of intraocular and periocular lymphomas and own experiences are presented. The treatment of primary vitreoretinal lymphomas (PVRL) is an interdisciplinary challenge and despite the apparently localized disease, systemic treatment concepts are necessary to reduce the high risk of secondary involvement of the central nervous system (CNS). Therefore, it is crucial that the substances used can penetrate the CNS, and protocols should be chosen in accordance with the treatment concepts for primary CNS lymphomas. The knowledge on the genetics and biology of ocular lymphomas generated by modern high throughput methods enable not only improved diagnostics using molecular methods but also provide rationales for targeted therapeutic approaches. Adeep understanding of the biological and molecular principles of intraocular and periocular lymphomas forms a basic prerequisite for precise diagnostics and the use of targeted systemic treatment.