title: Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study.
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 RT<30.6Gy (HR=4.17) were the only factors correlated with shorter PFS (P = .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.
authors: Hematology/Oncology, Sylvester Comprehensive Cancer Center, Miller School of Medicine.
authors: Amrita
authors: A
authors: Desai
authors: Ophthalmology, Bascom Palmer Eye Institute, and.
authors: Madhura G
authors: MG
authors: Joag
authors: Hematology/Oncology, Sylvester Comprehensive Cancer Center, Miller School of Medicine.
authors: Lazaros
authors: L
authors: Lekakis
authors: Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of Miami, Miami, FL.
authors: Jennifer R
authors: JR
authors: Chapman
authors: Hematology/Oncology, Sylvester Comprehensive Cancer Center, Miller School of Medicine.|Division of Hematopathology, Department of Pathology and Laboratory Medicine, University of Miami, Miami, FL.
authors: Francisco
authors: F
authors: Vega
authors: Departments of Biomedical Data Sciences and Statistics, Stanford University, Stanford, CA; and.
authors: Robert
authors: R
authors: Tibshirani
authors: Ophthalmology, Bascom Palmer Eye Institute, and.
authors: David
authors: D
authors: Tse
authors: Radiation Oncology, Sylvester Comprehensive Cancer Center, and.
authors: Arnold
authors: A
authors: Markoe
authors: Hematology/Oncology, Sylvester Comprehensive Cancer Center, Miller School of Medicine.|Department of Molecular and Cellular Pharmacology, Miller School of Medicine, University of Miami, Miami, FL.
authors: Izidore S
authors: IS
authors: Lossos
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D015331:Cohort Studies; D018572:Disease-Free Survival; D005134:Eye Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D011879:Radiotherapy Dosage; D012189:Retrospective Studies; D015996:Survival Rate; D055815:Young Adult