Ocular adnexal lymphoma: A single-institution retrospective study

César A Fernández; Roger K Henry; Carol L Shields; Jurij R Bilyk; Sara E Lally; Ralph C Eagle; Tatyana Milman

doi:10.4103/1319-4534.343368

Ocular adnexal lymphoma: A single-institution retrospective study

Saudi J Ophthalmol. 2022 Apr 18;35(3):230-238. doi: 10.4103/1319-4534.343368. eCollection 2021 Jul-Sep.

Authors

César A Fernández¹, Roger K Henry^{1

2}, Carol L Shields³, Jurij R Bilyk⁴, Sara E Lally³, Ralph C Eagle¹, Tatyana Milman^{1

5}

Affiliations

¹ Department of Pathology, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA.
² Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ, USA.
³ Ocular Oncology Service, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA.
⁴ Oculoplastic and Orbital Surgery Service, Wills Eye Hospital, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA.
⁵ Department of Pathology, Sidney Kimmel Medical College of Thomas Jefferson University, Philadelphia, PA, USA.

Abstract

Purpose: To characterize demographic, clinical, and histopathologic features of ocular adnexal lymphoma (OAL) at a single institution.

Methods: Retrospective review of all patients with pathologic diagnosis of OAL between 2015 and 2020.

Results: There were 133 patients with OAL, with a median age of 65 years (range 23-97) and a slight female predominance (male: female = 1:1.46), (n = 79, 59%). The majority of tumors were non-Hodgkin B-cell lymphomas (n = 131, 99%), most frequently Extranodal Marginal Zone B-Cell Lymphoma (EMZL, n = 93, 70%), followed by follicular lymphoma (n = 21, 16%), chronic lymphocytic leukemia/small lymphocytic lymphoma (n = 7, 5%), diffuse large B-cell lymphoma (n = 5, 4%), and mantle cell lymphoma (n = 5, 4%). The most frequently involved sites included the orbit (n = 85, 64%) and conjunctiva (n = 43, 32%). Information was available on oncologic staging in 78 (59%), treatment in 82 (62%), and follow-up in 75 (56%) patients. By the Ann-Arbor classification system, patients were classified as IE (54/78, 69%), IIE (9/78, 12%), IIIE (6/78, 8%), and IVE (9/78, 12%). The most common treatments included external beam radiotherapy (standard and ultra-low-dose) (48/82, 59%), biologics (22/82, 27%), and surgical excision with cryotherapy (14/82, 17%) (some patients had >1 therapy). Median follow-up time was 24 months (range 0-221 months). Recurrence was observed in 13% (10/75) with a median time to recurrence of 60 months (95% confidence interval 47-73 months). Excision with cryotherapy as a sole treatment modality was associated with earlier recurrence (P = 0.003).

Conclusion: In this largest single-center study of OAL, we found that most OAL were Ann-Arbor Stage IE EMZL, occurring in older patients with a female predominance. Early recurrence was noted in tumors treated with excision and cryotherapy alone.

Keywords: Ocular adnexal lymphoma; Ocular adnexal lymphoma pathology; ocular adnexal lymphoma recurrence; ocular adnexal lymphoma staging; ocular adnexal lymphoma treatment.