Long-term outcome of 487 patients with early-stage extra-nodal marginal zone lymphoma.
BACKGROUND
Localized early-stage extra-nodal marginal zone lymphoma (MZL) presents with heterogeneous organ involvement and is treated with various modalities, including resection, radiotherapy, and systemic therapy. We report the long-term outcome of a large cohort of extra-nodal MZL and assess the impact of patient and disease characteristics, organ site, and treatment strategy on disease control and survival.


METHODS
We identified 487 consecutive patients with stage IE or IIE MZL referred between 1992 and 2012 to Memorial Sloan Kettering Cancer Center. Pathology was reviewed by hematopathologists at our institution. Patient and disease factors as well as treatment types were analyzed for association with relapse-free survival, overall survival, and cumulative incidence of relapse.


RESULTS
Median follow-up after treatment was 4.7 years. Five-year relapse-free survival and overall survival were 60% and 89%, respectively. Cumulative incidence of disease-specific death at 5 years was 1.3%. Radiotherapy alone was the initial treatment in 50% of patients, followed by surgical resection (30%), observation (8%), immunotherapy (4%), and chemotherapy (2%). Initial treatment type, primary disease site, and number of involved sites were significant factors in multivariable analysis of relapse (all P < 0.05). When compared with stomach, MZL originating in other disease sites (HR > 2.0, P ≤ 0.001), except for thyroid, had higher risk of relapse. Strategies such as antibiotics or topical therapies were associated with higher risk of relapse when compared with radiation therapy (P < 0.001). Crude rate of transformation to pathologically confirmed large-cell lymphoma was 2% (11 patients).


CONCLUSIONS
Overall and cause-specific survival are high in early-stage extra-nodal MZL. Curative-intent treatment led to fewer relapses and reduced the need for salvage. Stomach cases had lower risk of relapse than other anatomic primary sites. This study supports the use of local therapies to treat stage IE and IIE MZL.
Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York.|Department of Radiation Medicine, Northwell Health, New York.|Hofstra Northwell School of Medicine, Hempstead, USA.
S
S
Teckie
Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York.|Department of Radiation Oncology, Cancer Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
S
S
Qi
Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York.
M
M
Chelius
Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York.
S
S
Lovie
Department of Epidemiology and Biostatistics, Memorial Sloan Kettering Cancer Center, New York.
M
M
Hsu
Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York.|Weill Cornell Medical College, New York, USA.
A
A
Noy
Lymphoma Service, Department of Medicine, Memorial Sloan Kettering Cancer Center, New York.|Weill Cornell Medical College, New York, USA.
C
C
Portlock
Department of Radiation Oncology, Memorial Sloan Kettering Cancer Center, New York.|Weill Cornell Medical College, New York, USA.
J
J
Yahalom
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D002648:Child; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D007167:Immunotherapy; D018442:Lymphoma, B-Cell, Marginal Zone / Q000453:epidemiology* / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000453:epidemiology* / Q000473:pathology / Q000628:therapy*; D009367:Neoplasm Staging; D011379:Prognosis; D016879:Salvage Therapy; D016896:Treatment Outcome; D055815:Young Adult
early stage; extranodal; marginal zone lymphoma; outcomes