Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci

• Published in: Blood Vol. 107; no. 2; pp. 467 - 472
• Main Authors: Rosado, Manuel F., Byrne, Gerald E., Ding, Feying, Fields, Kenneth A., Ruiz, Phillip, Dubovy, Sander R., Walker, Gale R., Markoe, Arnold, Lossos, Izidore S.
• Format: Journal Article
• Language: English
• Published: Washington, DC Elsevier Inc 15.01.2006; The Americain Society of Hematology; The American Society of Hematology
• Subjects: Adult; Aged; Aged, 80 and over; Biological and medical sciences; Chlamydophila psittaci - genetics; Chlamydophila psittaci - isolation & purification; Clinical Trials and Observations; Cohort Studies; Combined Modality Therapy; Conjunctival Neoplasms - microbiology; Conjunctival Neoplasms - pathology; Conjunctival Neoplasms - therapy; Eye Neoplasms - microbiology; Eye Neoplasms - pathology; Eye Neoplasms - therapy; Female; Humans; Lacrimal Apparatus Diseases - microbiology; Lacrimal Apparatus Diseases - pathology; Lacrimal Apparatus Diseases - therapy; Lymphoma, B-Cell - microbiology; Lymphoma, B-Cell - pathology; Lymphoma, B-Cell - therapy; Lymphoma, B-Cell, Marginal Zone - microbiology; Lymphoma, B-Cell, Marginal Zone - pathology; Lymphoma, B-Cell, Marginal Zone - therapy; Lymphoma, Large B-Cell, Diffuse - microbiology; Lymphoma, Large B-Cell, Diffuse - pathology; Lymphoma, Large B-Cell, Diffuse - therapy; Male; Medical sciences; Middle Aged; Neoplasm Staging; Ophthalmology; Orbital Neoplasms - microbiology; Orbital Neoplasms - pathology; Orbital Neoplasms - therapy; Prognosis; Psittacosis - microbiology; Psittacosis - pathology; Psittacosis - therapy; Risk Factors; Survival Rate; Treatment Outcome; Tumors and pseudotumors of the eye, orbit, eyelid, lacrimal apparatus; Human; Eye; Histopathology; Chlamydiaceae; Lymphoproliferative syndrome; Cohort study; Chlamydiales; Chlamydia psittaci; Bacteria; Malignant hemopathy; Ocular lymphoma; Lymphoma
• ISSN: 0006-4971; 1528-0020
• DOI: 10.1182/blood-2005-06-2332

Non-Hodgkin lymphomas are among the most common primary tumors occurring in the ocular adnexa. Herein, we present a 14-year single-institution experience in 62 patients with primary ocular adnexal lymphomas (OALs). Association with Chlamydia psittaci infection is examined in 57 tumor specimens. Extranodal marginal zone lymphoma (EMZL) was the most frequent histologic subtype (89%). The majority of patients with EMZL (84%) presented with stage E-extranodal (IE), however only 16% had an advanced stage. All stage IE patients were treated with local radiotherapy, whereas patients with disseminated disease received systemic therapy with or without local irradiation. All but 1 patient with EMZL achieved complete remission (CR). During a median follow-up of 52 months (range, 3-153 months), the estimated 5-year overall survival (OS) and freedom from progression (FFP) were 96% and 79%, 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. None of the OAL specimens harbored C psittaci DNA. Our study demonstrates that EMZLs, accounting for the majority of primary OALs, are characterized by an indolent natural history with frequent, continuous extranodal relapses. In South Florida, OALs are not associated with C psittaci infections.