Purpose:
To evaluate the approaches and sequence of diagnostic interventions used to confirm the presence of primary intraocular lymphoma (PIOL) in order to identify the most useful procedures for a timely diagnosis.
Methods:
A retrospective review was conducted of all PIOL patients referred to the NEI from 1994 to 2009. Medical records were reviewed for demographic data, time from onset of first ocular symptoms to definitive diagnosis, and method of ocular tissue sampling that yielded a positive histopathologic diagnosis. Procedures considered to be potentially diagnostic and if positive, would lead to initiation of treatment included: lumbar puncture (LP), pars plana vitrectomy (PPV), vitreous tap, anterior chamber aspiration, chorioretinal biopsy and brain biopsy.
Results:
A total of 23 patients were identified with PIOL. Twenty patients (87.0%) reported decreased vision or floaters at initial presentation. The mean duration of symptoms before diagnosis was 14.2 months (range 1-96 months). Patients required an average of 2.04 procedures to establish the correct diagnosis (range 1-4; median 2.0). Of these, 8 (34.8%) had the definitive results on the first procedure and 15 (65.4%) had at least one false negative procedure. The diagnosis was made by PPV in 11 cases (47.8%). Brain biopsy was positive in 4 patients, vitreous tap in 3, LP in 3, and chorioretinal biopsy in 2. Of the 8 patients diagnosed correctly after the first procedure, vitreous biopsy (50%) brain biopsy (25%) and LP (25%) were the initial diagnostic method chosen. PPV was positive in 11 of the 17 cases (64.7%). In the 6 cases with a false negative PPV, the second diagnostic procedure (PPV, vitreous tap, or chorioretinal biopsy) was always successful in confirming the diagnosis.
Conclusions:
The diagnostic evaluation of PIOL continues to be a challenge. It can take multiple diagnostic procedures to confirm the diagnosis. A high level of clinical suspicion and proper handling of samples is essential for accurate diagnosis. Brain and chorioretinal biopsy were always successful but rarely employed due to their invasive nature. PPV was the most useful initial test for histopathologic diagnosis.
Keywords: oncology • immunohistochemistry • retina