Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study
Open Access
- 19 January 2017
- journal article
- Published by American Society of Hematology in Blood
- Vol. 129 (3), 324-332
- https://doi.org/10.1182/blood-2016-05-714584
Abstract
While primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (POAML) is the most common orbital tumor, there are large gaps in knowledge of its natural history. We conducted a retrospective analysis of the largest reported cohort, consisting of 182 patients with POAML, diagnosed or treated at our institution to analyze long-term outcome, response to treatment, and incidence and localization of relapse and transformation. The majority of patients (80%) presented with stage I disease. Overall, 84% of treated patients achieved a complete response after first-line therapy. In patients with stage I disease treated with radiation therapy (RT), doses ≥30.6 Gy were associated with a significantly better complete response rate (P = .04) and progression-free survival (PFS) at 5 and 10 years (P < .0001). Median overall survival and PFS for all patients were 250 months (95% confidence interval [CI], 222 [upper limit not reached]) and 134 months (95% CI, 87-198), respectively. Kaplan-Meier estimates for the PFS at 1, 5, and 10 years were 91.5% (95% CI, 86.1% to 94.9%), 68.5% (95% CI, 60.4% to 75.6%), and 50.9% (95% CI, 40.5% to 61.6%), respectively. In univariate analysis, age >60 years, radiation dose, bilateral ocular involvement at presentation, and advanced stage were significantly correlated with shorter PFS (P = .006, P = .0001, P = .002, and P = .0001, respectively). Multivariate analysis showed that age >60 years (hazard ratio [HR] 2.44) and RTP = .01 and P = .0003, respectively). We demonstrate that POAMLs harbor a persistent and ongoing risk of relapse, including in the central nervous system, and transformation to aggressive lymphoma (4%), requiring long-term follow-up. Publisher9s Note: There is an Inside Blood Commentary on this article in this issue. Key Points POAML (specifically Ann Arbor stage I disease) has an excellent clinical outcome, with only a few patients succumbing to lymphoma. POAML patients face a continuous risk of distant relapse, including in the central nervous system, and transformation to aggressive lymphoma.Keywords
This publication has 49 references indexed in Scilit:
- Antibiotic therapy in nongastrointestinal MALT lymphoma: a review of the literatureBlood, 2013
- Molecular and genomic aberrations in Chlamydophila psittaci negative ocular adnexal marginal zone lymphomasAmerican Journal of Hematology, 2013
- Efficacy of low dose radiotherapy for primary orbital marginal zone lymphomaLeukemia & Lymphoma, 2012
- Chlamydophila Psittaci Eradication With Doxycycline As First-Line Targeted Therapy for Ocular Adnexae Lymphoma: Final Results of an International Phase II TrialJournal of Clinical Oncology, 2012
- Treatment Effects and Sequelae of Radiation Therapy for Orbital Mucosa-Associated Lymphoid Tissue LymphomaInternational Journal of Radiation Oncology*Biology*Physics, 2011
- Primary ocular adnexal mucosa‐associated lymphoid tissue lymphoma (MALT): single institution experience in a large cohort of patientsBritish Journal of Haematology, 2010
- Extranodal marginal zone lymphoma of the ocular adnexaBlood, 2009
- Low-Grade Non-Hodgkin LymphomasSeminars in Radiation Oncology, 2007
- Orbital marginal zone B-cell lymphoma of MALT: Radiotherapy results and clinical behaviorInternational Journal of Radiation Oncology*Biology*Physics, 2006
- The Clinical Characteristics and Treatment Results of Ocular Adnexal LymphomaKorean Journal of Ophthalmology, 2006