Abstract
Purpose :
Primary ocular adnexal lymphoma (POAL) is the most common malignancy of the eye. The most frequent subtypes of POAL are extranodal marginal zone lymphoma (ENMZL) of mucosa-associated lymphoid tissue (MALT). To improve the diagnosis and quality of treatment in a patient with POAML, we performed a retrospective multicenter cohort study to assess the characteristics and treatment outcomes of patients with primary ocular adnexal MALT lymphoma in Thailand.
Methods :
Patients diagnosed with POAML between January, 2004 and December, 2016 who had been followed for at least one year from Chiang Mai University Hospital and King Chulalongkorn Memorial Hospital, Thailand were included. The demographic and response rates were analyzed with descriptive statistics. Probabilities of overall survival and progression-free survival were estimated by using the Kaplan Meier method and using log-rank test for survival comparison.
Results :
A total number of 121 patients were identified of which 52.9% were female. A median age was 58.0 years (Range, 22-86). The most common presenting symptom was orbital mass (71.1%), while 25.3% of patients presented with proptosis. The sites of origin were as follows: orbit (46.3%), lacrimal (34.7%), conjunctiva (9.9%) and eyelid (8.3%). Half of the patients had disease stage I (n=59, 56.2%) while the rest of patients had stage IV (20%). The majority of the patients (73.3%) had a low-risk international prognosis index (IPI). Radiotherapy is the main treatment for a limited stage (66.7% in stage I and 56.5% in stage II). The overall response rate was 100% with complete response rate (CR) of 80%, 77.3% and 64.7% for stage I, II and IV respectively. The 5-year PFS an OS were 66.1% and 94.0%, respectively. For those patients with a limited stage of disease, radiotherapy significantly improved PFS than non-radiotherapy-containing treatment (5-yrs PFS 80.3% VS 48.2%, P=0.013).
Conclusions :
POAML is the most common type of POAL in Thailand with a good response to treatment and excellent long-term outcome. Radiotherapy has a main role in disease control, contributing to an improvement of survival outcome this group of patients.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.