gms | German Medical Science

Background: Primary intraocular lymphoma (PIOL, synonym primary vitroretinal lymphoma) is a manifestation of primary CNS lymphoma (PCNSL), which may occur before the CNS manifestations, simultaneously with PCNSL or at progression/relapse. It is a very rare disorder, and its diagnostic and therapeutic management is yet to be defined. The objectives of this analysis is to present the most important aspects of the diagnostics and therapy of PIOL and formulate management recommendations.

Methods: The most recent German and English language literature was reviewed and data were analyzed for presentation. For the meeting, data from the ongoing prospective German PIOL registry (PIOL-R) will additionally be analysed.

Results: The diagnosis of PIOL is often difficult as it usually mimics chronic intermediate or posterior uveitis. To make the definite diagnosis, various procedures, e.g. immunohistochemistry, cytology, pathology, molecular pathology and cytokine analysis (interleukin 10) from ocular specimens are required. Evaluation for central nervous system involvement including MRI of the cerebrum and lumbar puncture should be performed. The prognosis is relatively poor, particularly when the CNS is involved. Optimal therapy has not yet been established. For isolated vitreoretinal manifestations local therapy, such as intraocular methotrexate (MTX) or rituximab or radiation is possible; however, there is a very high frequency of CNS relapse. Systemic high-dose methotrexate- and/or cytarabine-based chemotherapy analogous to PCNSL treatment is an alternative option, and is the treatment of choice in patients with simultaneous CNS and vitreoretinal involvement.

Conclusions: Data on PIOL is still scarce. More awareness is needed to make the diagnosis early and to prevent irreversible deficits and probably CNS relapse. To gain more knowledge on this rare disease, inclusion of patients in the prospective registry, currently being conducted in Germany, is desirable.