Management of primary intraocular lymphoma (PIOL): results from the prospective German PIOL Registry (PIOL-R)

Abstract

Purpose: Primary intraocular lymphoma (PIOL) is a very rare disorder, and the optimal treatment is yet to be defined. Here, we report on clinical characteristics, treatment and outcome of patients with PIOL enrolled in the prospective German PIOL registry (PIOL-R).

Methods: Patient data in this prospective, non-interventional multicenter study were compiled by standardized questionnaires sent to Ophthalmology and Hematology/Oncology departments in Germany. Only immunocompetent patients were eligible and diagnosis had to be confirmed histologically or cytologically.

Results: Twenty patients (12 female, median age 67 years, median Karnofsky performance status 90%) were included between August 2008 and August 2013. Median follow-up was 20.2 months. Median time from onset of symptoms to PIOL diagnosis was 5 (range, 1-12) months. Diffuse large B-cell histology was found in all patients. Eleven patients had prior (n=3), concomitant (n=3), and/or subsequent (n=5) parenchymal brain involvement. First-line treatment included high-dose Methotrexate (MTX)- or Ifosfamide-based systemic chemotherapy with or without Rituximab in 17 (including high-dose chemotherapy [HDCT] with autologous stem cell transplantation [ASCT] in 2), intraocular (i.o.) chemotherapy with MTX (n=2) and/or Rituximab (n=8) in 9, and ocular radiation in one patient(s). Two patients received prophylactic intrathecal treatment with MTX but none had whole-brain irradiation. The PIOL remission rate was 100% with complete remission in 14/18 evaluable patients and partial remission in 4 patients. Median progression-free survival was 8.1 months. Overall survival has not yet been reached. Relapses were located in the brain (n=8), eye (n=1), eye/brain (n=3) and eye/meninges (n=1) and were treated with systemic chemotherapy in 10 (including HDCT with ASCT in 3), systemic and i.o. chemotherapy in 1, and whole brain/spine radiotherapy in 2 patient(s).

Conclusions: Although the awareness of PIOL and diagnostic possibilities have improved over the past two decades, time from symptom presentation to diagnosis seems to remain at previously reported levels. There appears to be a shift from local ocular treatments and radiation to systemic therapy as compared to anecdotal data with promising response and survival rates. However, relapse rates remain high.

Keywords: 744 tumors • 745 uvea • 452 choroid