Evaluation of vitrectomy specimens and chorioretinal biopsies in the diagnosis of primary intraocular lymphoma in patients with Masquerade syndrome

Abstract

The diagnosis of PIOL is often extremely difficult, requiring sufficient rapidly transported good-quality material, and experienced interpretation. Although cytological examination of vitreal aspirates remains the gold standard in diagnosis, examination of chorioretinal biopsies increase the reliability of diagnosing or excluding a PIOL that involves the retina or choroid. Most PIOL are DLBCL with an immunophenotype suggesting a cellular origin from germinal centre cells.

The pathologist's perspective on vitreous opacities

“…[4][5][6][7]9,10,20,21,26,[30][31][32][33][34][35] These include: (1) vitreous 'filtration'; (2) a celloidin bag technique; (3) cytospin and (4) cell block preparation. We are most familiar with the latter two methods, whereby the vitreous is spun at 500 r.p.m.…”

“…Vitreous and aqueous samples may be accompanied by specimens obtained from tissues such as retina and choroid. 20,36 These solid specimens are usually fixed in buffered formalin, and processed in paraffin using standard procedures.…”

“…Should sufficient material be available for examination, investigation of the vitreous specimens for rearrangements of the immunoglobulin heavy chain gene using PCR provides further evidence of the neoplastic nature of the lymphocytic infiltrate in retinal lymphoma. 17,18,20,22,25,35,49,51 Biochemical analysis of the vitreous specimen for interleukin ratios (IL10 : IL6) may also support the diagnosis of retinal lymphoma. [52][53][54] If possible, retinal lymphoma should be distinguished from other types of intraocular lymphoma, namely primary uveal lymphoma and secondary (metastatic) intraocular lymphoma.…”

Primary Intraocular Diffuse Large B-cell Lymphoma: Diagnostic Difficulties in Deep Retinal Infiltrations with Vitritis

“…Differential diagnosis should include infectious and non-infectious aetiologies, particularly the common masqueraders such as sarcoidosis, tuberculosis, viral retinitis and syphilis [2]. This rare disease should be suspected in all patients with uveitis resistant to corticosteroid therapy and in elderly patients with a recent onset of vitritis [3]. Patients typically complain of decreased vision, painless blurred vision and floaters, most likely secondary to the chronic vitritis and subretinal lesions.…”

Primary Intraocular Lymphoma

“…163,183 The mean age of presentation is in the fifth and sixth decades of life. 19,51,85,163,213 Some reports suggest a sex bias, with women more commonly affected than men by 2:1 15,21,163 or even greater. 27,168 More recently there appears to have been an increase of reported 6 cases in men.…”