Ocular Reticulum Cell Sarcoma

Biopsy techniques and yields in diagnosing primary intraocular lymphoma

“…Prior to the advent of pars plana vitrectomy in the 1970s pioneered by Machemer [10][11][12][13][14][15], enucleation [16,17] was the first surgical procedure employed by ophthalmic surgeons to make a diagnosis of the so called reticulum cell sarcoma (as PIOL was known prior to the 1970s) [8,17,18]. Enucleation could be performed in patients' eyes in which there was no possibility of restoring vision (vision worse than count fingers) due to massive involvement or in eyes that had become intractably painful.…”

“…It is important to realize that, if a patient is suspected of having PIOL, a lumbar puncture with cytologic analysis of the cerebrospinal fluid (CSF) should be performed because of the high possibility of brain involvement in PIOL [1,3,[7][8][9]. In addition, patients should also receive a brain magnetic resonance imaging (MRI) scan to determine whether lesions are present in the absence of obvious lymphoma cells in the CSF.…”

Intraocular Lymphoma

“…[4][5][6][7][8] The most common subjective symptoms are painless decreased vision and "floaters." The typical clinical profile is an elderly patient with uveitis that is refractory to treatment.…”

“…PCNSLO may be unilateral or bilateral on initial presentation, but ultimately, 80% to 90% of patients will have bilateral involvement. [4][5][6][7][8]11 Intracranial disease occurs in 56% to 85% of patients with ocular disease, and recent estimates suggest that 15% to 25% of patients who present with CNS disease will have ocular disease, hence the distinction between PCNSL and PCNSLO. [4][5][6][7][8] Data regarding the cause of PCNSLO are not conclusive at this time, although PCNSLO appears to occur with increased frequency in persons who are severely immunosuppressed (see below).…”

“…9 It typically affects elderly patients, with reported series having mean ages in the 60s. [4][5][6][7][8] The youngest reported patient was 3 years old, and the initial case occurred in a 27-year-old patient. 10,11 Women are affected up to twice as often as men, and there is no racial predilection.…”