Blood Res.  2018 Dec;53(4):307-313. 10.5045/br.2018.53.4.307.

Clinical presentation and treatment outcomes of primary ocular adnexal MALT lymphoma in Thailand

• Seresirikachorn K
• Norasetthada L
• Ausayakhun S
• Apivatthakakul A
• Tangchittam S
• Pruksakorn V
• Wudhikarn
• Wiwatwongwana D

• KMID: 2429305
• DOI: http://doi.org/10.5045/br.2018.53.4.307

Abstract

BACKGROUND
Primary ocular adnexal extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (POML) is the most common subtype of lymphoma involving the eyes in Thailand. We sought to assess the characteristics and treatment outcomes of patients with POML in Thailand.
METHODS
We retrospectively reviewed patient data and included patients diagnosed with POML between January 2004 and December 2016 at Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand. We collected and analyzed patients' clinical characteristics and treatment outcomes.
RESULTS
Among 146 patients with lymphoma involving the eyes, 121 (82%) were diagnosed with POML. Sixty-four (52.9%) were women with median age 58 (range, 22-86) years. The most common presenting symptom was orbital mass (71.1%). Common sites of origin were the orbit (46.3%) and lacrimal gland (34.7%). At presentation, 22.3% of patients had bilateral eye involvement. About half of patients had stage I disease (N=59, 56.2%) and 20% had stage IV. Most patients (73.3%) had a low-risk International Prognostic Index. Radiotherapy was the main treatment for patients with limited-stage disease (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rates 80%, 77.3%, and 64.7% for stages I, II, and IV, respectively. Five-year progression-free survival (PFS) and overall survival were 66.1% and 94.0%, respectively. For patients with limited-stage disease, radiotherapy significantly improved PFS compared with treatment not involving radiotherapy (5-year PFS 89.9% vs. 37.3%, P=0.01).
CONCLUSION
We revealed that POML has good response to treatment, especially radiotherapy, with excellent long-term outcome.

Keyword

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue; Ocular adnexal lymphoma; MALT lymphoma; Radiotherapy

MeSH Terms

Disease-Free Survival
Female
Humans
Lacrimal Apparatus
Lymphoma
Lymphoma, B-Cell, Marginal Zone*
Orbit
Radiotherapy
Retrospective Studies
Thailand*

Figure

• 

  Fig. 1 Kaplan-Meier curve of progression-free survival (PFS) in patients with primary ocular adnexal MALT lymphoma (A) and primary ocular adnexal MALT lymphoma (B).

• 

  Fig. 2 Kaplan-Meier curve of progression-free survival (PFS) in patients with primary ocular adnexal MALT lymphoma, according to Ann Arbor stage. (A) Five-year PFS 77.4% versus 52.5% for patients with limited- versus advanced-stage disease (P=0.063). (B) Five-year OS 96.0% versus 81.7% for patients with limited- versus advanced-stage disease (P=0.123).

• 

  Fig. 3 (A) Kaplan-Meier progression-free survival (PFS) between radiotherapy and non-radiotherapy-based treatment in patients with limited-stage primary ocular adnexal MALT lymphoma. Five-year PFS 89.9% and 52.9%, respectively (P=0.007). (B) Kaplan-Meier progression-free survival (PFS) comparison between radiotherapy-containing therapy and non-radiotherapy-containing therapy in patients with advanced-stage primary ocular adnexal MALT lymphoma. Five-year PFS 66.7% and 49.5%, respectively (P=0.52).

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