[New systemic treatment approach for primary lymphoma of the orbit and the ocular adnexa].

Background: Orbital lymphomas are the most frequently occurring malignant tumor entities in this region. They are less frequently also localized in the ocular adnexa and show a high entity-specific heterogeneity regarding the treatment and prognosis.

Objective: This article gives an overview of the heterogeneity of symptoms of orbital lymphoma and lymphoma of the ocular adnexa and focuses on new systemic options for treatment of this disease.

Material and methods: For this article a systematic search of the PubMed databank was carried out and the practical knowledge of the authors was taken into account.

Results and conclusion: Orbital lymphoma and lymphoma of the ocular adnexa show a high variability in the clinical symptoms and therefore require a comprehensive ophthalmological examination of the affected patients. Histopathologically assisted diagnostics after an incisional biopsy are essential for the classification into the various lymphoma entities. This is necessary for developing and carrying out a targeted treatment concept consisting of local and systemic treatment modalities. Especially for the new systemic treatment approaches, in addition to the histopathological classification of the lymphoma entity it is also necessary to analyze the molecular features in order to efficiently implement the new highly specific medications. Finally, a close interdisciplinary cooperation of all disciplines involved is absolutely necessary for the best possible treatment, including ophthalmology, hemato-oncology, radiotherapy, radiology, pathology and psycho-oncology. Depending on the localization of the tumor, inclusion of otorhinolaryngology, oral and maxillofacial surgery and/or neurosurgery can be necessary.