Augenbefall und Systemerkrankung – periokuläre und intraokuläre Lymphome

Background: Ocular lymphoma is classified as intraocular lymphoma and periocular lymphoma based on the anatomic location. Intraocularly, the uvea with its structures can be affected or the retina in combination with the vitreous body. Periocular lymphoma can occur in the orbit, the conjunctiva, the lacrimal apparatus, or the eye lid. The differentiation between primary lymphomas of the ocular region and systemic disease is of exceptional importance. Over the last few decades, the incidence has risen constantly in Western countries. Aim: An overview of the heterogeneous manifestations, diagnostics, treatment strategies, prognosis, and aftercare of patients with ocular lymphoma is given. Materials and methods: This review is based on a selective literature search of the MEDLINE database and on the authors’ personal experience with this disease. Results: Depending on the location of the tumor, the signs and symptoms can be very diverse. Diagnosis is based on biopsy and subsequent cytologic/histopathologic and when appropriate molecular pathologic examinations of the tissue. Radiation therapy and systemic approaches are the most common treatment options. Prognosis is dependent on location, subtype of lymphoma, and extent of systemic involvement. Conclusion: The diagnosis, treatment and aftercare of ocular lymphoma requires a multidisciplinary approach between ophthalmology, hemato-oncology, radiotherapy, neurology, neurosurgery, oral and maxillofacial surgery, otorhinolaryngology, dermatology, radiology, pathology, and psycho-oncology. The ophthalmologist often acts as the primary contact person in this disease.