Characteristics and treatment outcomes of patients with primary ocular adnexal lymphoma in Northern Thailand

  • Kasem Seresirikachorn Department of Ophthalmology, Faculty of Medicine, Chiang Mai University, Chiang Mai, Thailand
  • Sakarin Ausayakhun
  • Damrong Wiwatwongwana
  • Ponsak Mahanupab
  • Teerada Daroontum
  • Lalita Norasetthada
Keywords: extranodal marginal zone lymphoma of mucosal-associated lymphoid tissue, ocular adnexal lymphoma, radiotherapy, treatment modalities


Purpose: To assess the characteristics and treatment outcomes of patients with primary ocular adnexal lymphoma (OAL) in Northern Thailand.

Design: Retrospective cohort study.

Methods: Data was collected from electronic medical records and operative notes from Chiang Mai University Hospital between January 2009 and December 2014. All available tissue biopsies of 54 patients were reviewed by agreement of two pathologists. The clinical characteristics and treatment outcomes were collected and analyzed.

Results: A total number of 54 patients were identified of which 57.4% were female. The median age was 61.0 years (range, 4-86). The most common subtype of lymphoma was extranodal marginal zone lymphoma (ENMZL) of mucosa-associated lymphoid tissue (MALT) (n = 46, 85.2%). Seventy-five percent of the patients presented with a mass at the ocular adnexa, while 14.8% of the patients presented with proptosis. The sites of origin were as follows: lacrimal (46.3%), orbit (31.5%), conjunctiva (13%) and eyelid (7.4%). Two-thirds of the patients had Ann-Arbor Stage I, while 22% of patients had Stage IV. The majority of the patients (68.1%) had a low-risk international prognosis index (IPI). Treatment modalities involved field radiation (IFRT, 50%), chemotherapy (31.6%), combined chemoradiotherapy (7.9%) and surgical resection (10.5%). The overall response rate was 100% with a complete response rate of 77.8%. In patients with low-grade lymphoma, including MALT lymphoma, the 3-year progression-free survival (PFS) and overall survival were 69.9% and 92.5%, respectively.

Conclusion: ENMZL of MALT was the major subtype of primary OAL. Radiotherapy was an effective treatment for the lower stages of disease providing a high response rate and encouraging survival outcomes.


Margo CE, Mulla ZD. Malignant tumors of the orbit: analysis of the Florida Cancer Registry. Ophthalmology. 1998;105:185-190.

Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphoma. Cancer. 1972;29:252-260.

White WL, Ferry JA, Harris NL, Grove AS, Jr. Ocular adnexal lymphoma: a clinicopathologic study with identification of lymphomas of mucosa-associated lymphoid type. Ophthalmology. 1995;102:1994-2006.

McKelvie PA, McNab A, Francis IC, Fox R, O’Day J. Ocular adnexal lymphoproliferative disease: a series of 73 cases. Clin Exp Ophthalmol. 2001;29:387-393.

Ferreri AJ, Dolcetti R, Du MQ, et al. Ocular adnexal MALT lymphoma: an intriguing model for antigen-driven lymphomagenesis and microbial-targeted therapy. Ann Oncol. 2008;19:835-846.

Rosado MF, Byrne GE Jr, Ding F, et al. Ocular adnexal lymphoma: a clinicopathologic study of a large cohort of patients with no evidence for an association with Chlamydia psittaci. Blood. 2006;107:467-472.

Le QT, Eulau SM, George TI, et al. Primary radiotherapy for localized orbital MALT lymphoma. Int J Radiat Oncol Biol Phys. 2002;52:657-663.

Tsang, RW, Gospodarowicz MK, Pintilie M, et al. Localized mucosa-associated lymphoid tissue lymphoma treated with radiation therapy has excellent clinical outcome. J Clin Oncol. 2003;21:4157-4164.

Tulvatana W, Paisuntornsug T, Keelawat S, Sansopha L, Karnsawai S. Malignant tumors of the eye and ocular adnexa at King Chulalongkorn Memorial Hospital: a twelve-year review (1988-1999). Chulalongkorn Med J. 2001;45(4):283-293.

Na Pombejara F, Tulvatana W, Pungpapong K. Malignant tumours of the eye and ocular adnexa in Thailand: a six-year review at King Chulalongkorn Memorial Hospital. Asian Biomed. 2009;3(5):551-555.

Jenkins C, Rose GE, Bunce C, et al. Histological features of ocular adnexal lymphoma (REAL classification) and their association with patient morbidity and survival. Br J Ophthalmol. 2000;84:907-913.

Meunier J, Lumbroso-Le RL, Vincent-Salomon A, et al. Ophthalmologic and intraocular non-Hodgkin’s lymphoma: a large single center study of initial characteristics, natural history, and prognostic factors. Hematol Oncol. 2004;22:143-158.

Ferry JA, Fung CY, Zukerberg L, et al. Lymphoma of the ocular adnexa: a study of 353 cases. Am J Surg Pathol. 2007;31:170-184.

Portell CA, Aronow ME, Rybicki LA, Macklis R, Singh AD, Sweetenham JW. Clinical characteristics of 95 patients with ocular adnexal and uveal lymphoma: treatment Outcomes in Extranodal Marginal Zone Subtype. Clin Lymphoma Myeloma Leuk. 2014;14(3):203-210.

Sniegowski MC, Robert D, Bakhoum M, et al. Ocular adnexal lymphoma: validation of American Joint Committee on Cancer seventh edition staging guidelines. Br J Ophthalmol. 2014;98:1255-1260.

Asadi-Amoli F, Nozarian Z, Bonaki HN, Mehrtash V, Entezari S. Clinicopathologic assessment of ocular adnexal lymphoproliferative lesions at tertiary eye hospital in Iran. Asian Pac J Cancer Prev. 2016;17(8):3727-3731.

Coupland SE, Hellmich M, Auw-Haedrich C, Lee WR, Anagnostopoulos I, Stein H. Plasmacellular differentiation in extranodal marginal zone B cell lymphomas of the ocular adnexa: an analysis of the neoplastic plasma cell phenotype and its prognostic significance in 136 cases. Br J Ophthalmol. 2005;89:352-359.

Tanimoto K, Kaneko A, Suzuki S, et al. Primary ocular adnexal MALT lymphoma: a long-term follow-up study of 114 patients. Jpn J Clin Oncol. 2007;37:337-344.

Bayraktar S, Bayraktar UD, Stefanovic A, Lossos IS. Primary ocular adnexal mucosa-associated lymphoid tissue lymphoma (MALT): single institution experience in a large cohort of patients. Br J Haematol. 2010;152:72-80.

Ferreri AJ, Ponzoni M, Guidoboni M, et al. Evidence for an association between Chlamydia psittaci and ocular adnexal lymphomas. J Natl Cancer Inst. 2004;96:576-584.

Ferreri AJ, Guidoboni M, Ponzoni M, et al. Regression of ocular adnexal lymphoma after Chlamydia psittaci-eradicating antibiotic therapy. J Clin Oncol. 2005;23:5067-5073.

Conconi A, Martinelli G, Thieblemont C, et al. Clinical activity of rituximab in extranodal marginal zone B-cell lymphoma of MALT type. Blood. 2003;102:2741-2745.

Lossos IS, Morgensztern D, Blaya M, Alencar A, Pereira D, Rosenblatt J. Rituximab for treatment of chemoimmunotherapy naive marginal zone lymphoma. Leuk Lymphoma. 2007;48:1630-1632.

Raderer M, Jäger G, Brugger S, et al. Rituximab for treatment of advanced extranodal marginal zone B cell lymphoma of the mucosa-associated lymphoid tissue lymphoma. Oncology. 2003;65:306-310.

Nückel H, Meller D, Steuhl K-P, Dührsen U. Anti-CD20 monoclonal antibody therapy in relapsed MALT lymphoma of the conjunctiva. Eur J Haematol. 2004;73:258-262.

Ferreri AJM, Ponzoni M, Martinelli G, et al. Rituximab in patients with mucosal-associated lymphoid tissue-type lymphoma of the ocular adnexa. Haematologica. 2005;90:1578-1579.

Benetatos L, Alymara V, Asproudis I, Bourantas KL. Rituximab as first line treatment for MALT lymphoma of extraocular muscles. Ann Hematol. 2006;85:625-626.

Heinz C, Merz H, Nieschalk M, Mueller-Miny H, Koch P, Heiligenhaus A. Rituximab for the treatment of extranodal marginal zone B-cell lymphoma of the lacrimal gland. Br J Ophthalmol. 2007;91:1563-1564.

How to Cite
Seresirikachorn, K., Ausayakhun, S., Wiwatwongwana, D., Mahanupab, P., Daroontum, T., & Norasetthada, L. (2020). Characteristics and treatment outcomes of patients with primary ocular adnexal lymphoma in Northern Thailand. Asian Journal of Ophthalmology, 17(1), 86-97.