Ocular Adnexal Lymphoma: A Clinicopathological Study of a Large Cohort of Patients with No Evidence for an Association with Chlamydia psittaci.

Abstract

Ocular adnexal lymphomas (OALs) represent 6% of all primary extranodal NHL. However, they are among the most common primary tumors occurring in the ocular adnexa (26%). The etiology of OALs and the reason for the predominance of extranodal marginal zone lymphoma (EMZL) in this anatomical location are unknown. Ferreri et. al (J Natl Cancer Inst 2004; 96:586) recently reported the presence of C psittaci DNA in 80% of the analyzed Italian ocular adnexa EMZL cases. Herein, we reviewed our 14-year single institution experience in a large cohort of patients with primary OALs and examined the association with C psittaci infection in South Florida. Clinicopathological characteristics, response to therapy, systemic dissemination and patients' survival were analyzed in 62 patients with primary OALs treated and followed-up at the Sylvester Comprehensive Cancer Center. EMZL was the most frequent histological subtype (89% of OALs). Majority of EMZL patients (84%) presented with stage IE, however only 16% had an advanced stage. All stage IE patients were treated with local radiotherapy, while patients with disseminated disease received systemic therapy with or without local irradiation. All but one EMZL patient achieved CR. During a median follow up of 52 months (range 3–153), the estimated 5-year OS and FFP were 96% (95% CI: 90 to 100%) and 79% (95% CI: 65 to 92%), respectively. During the follow-up, 22% of patients relapsed, mainly in extranodal sites and 4% transformed to diffuse large B-cell lymphoma. None of the patients exhibited local orbital failure in the radiation field. To analyze the association between C psittaci infection and OALs in our patient population, C psittaci touchdown enzyme time release-PCR was used in 57 tumor specimens. DNA was extracted from 49 specimens of EMZL of ocular adnexa, 8 specimens of non- EMZL of ocular adnexa and 2 specimens from patients with reactive ocular lymphoid hyperplasia. C psittaci DNA was not detected in any of the 57 tumor DNA samples positive for either beta-globin or ApoE DNA quality control amplicons, while control reactions using DNA prepared form C psittaci strain 6 BC yielded amplicon of expected size. It is thus possible that there is a regional variation in EMZL etiology and association with infection pathogens. Additional studies evaluating the association between C psittaci and EMZL of ocular adnexa in other geographical regions are granted.