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LONG-TERM OUTCOME OF 490 PATIENTS WITH EARLY-STAGE EXTRA-NODAL MARGINAL ZONE LYMPHOMA
Author(s): ,
Joachim Yahalom
Affiliations:
Radiation Oncology,Memorial Sloan-Kettering Cancer Center,New York,United States
,
Sewit Teckie
Affiliations:
Radiation Oncology,Memorial Sloan-Kettering Cancer Center,New York,United States
,
Shunan Qi
Affiliations:
Radiation Oncology,Memorial Sloan-Kettering Cancer Center,New York,United States
,
Meier Hsu
Affiliations:
Epidemiology and Biostatistics,Memorial Sloan-Kettering Cancer Center,New York,United States
,
Shona Lovie
Affiliations:
Radiation Oncology,Memorial Sloan-Kettering Cancer Center,New York,United States
,
Monica Chelius
Affiliations:
Radiation Oncology,Memorial Sloan-Kettering Cancer Center,New York,United States
,
Ariela Noy
Affiliations:
Medicine,Memorial Sloan-Kettering Cancer Center,New York,United States
Carol Portlock
Affiliations:
Medicine,Memorial Sloan-Kettering Cancer Center,New York,United States
(Abstract release date: 05/21/15) EHA Library. Yahalom J. 06/12/15; 103173; S108 Disclosure(s): Memorial Sloan-Kettering Cancer Center
Radiation Oncology
Joachim Yahalom
Joachim Yahalom
Contributions
Abstract
Abstract: S108

Type: Oral Presentation

Presentation during EHA20: From 12.06.2015 12:00 to 12.06.2015 12:15

Location: Room A7

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, infrequently, 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.

Aims
To study patient characteristics and long-term outcome of a large cohort of patients with early-stage localized extra-nodal marginal zone lymphoma

Methods
We identified 490 consecutive patients with stage IE or IIE MZL referred between 1992 and 2012 to Memorial Sloan Kettering Cancer Center. Pathology was confirmed by hematopathologists at our institution. Patient and disease factors as well as treatment types were analyzed for association with relapse-free survival (RFS), overall survival (OS), and cumulative incidence of relapse.

Results

Median follow-up was 4.8 years. Median patient age was 60 years and 57% were female. Ann-Arbor stage was IE in 89%. Most common sites were stomach (32%), orbit (14%), lung (12%), skin (12%), and parotid (5%). Radiotherapy alone (RT) was the initial treatment in 50% of patients, followed by surgical resection (30%), observation (9%), immunotherapy (4%), and chemotherapy (2%).

Five- year OS and RFS were 90% and 64%, respectively; 10-year OS and RFS were 73% and 45%. Disease-specific death was 1.3% at 5 years and 1.8% at 10 years. Cumulative incidence of progression/relapse was 29% by 5 years and 39% by 10 years. Amongst the 384 patients with complete response (CR), 99 patients experienced relapse. On multivariable analysis, initial treatment type and primary disease site were independently associated with RFS and relapse (all p<0.005). All disease sites (HR>2.0, p≤0.01) except for thyroid (p=0.8) had worse RFS relative to stomach.  Compared with RT, chemotherapy or immunotherapy had worse RFS (HR 2.2, p=0.004) while surgery was no different (p=0.52). After RT, only 11 patients experienced in-field failure, with a 5-year cumulative incidence of 2.4%. Most common location of relapse after CR was distant; relapses were also observed in paired untreated organs, such as the orbit, salivary gland, and breast. Crude rate of transformation to pathologically-confirmed large-cell lymphoma was 2% (11 patients). Second tumors in irradiated sites developed in 3 patients: 2 of these were breast ductal carcinoma in situ cured with surgical resection.



Summary
Overall and cause-specific survival are excellent in early-stage extra-nodal MZL. Treatment with RT or surgery was associated with longer RFS and reduced the need for salvage. Relapses are common after initial remission, and most frequently occur in distant sites. Transformation to large cell lymphomas is rare. Stomach cases are less likely to relapse than other anatomic primary sites, perhaps in part because the entire organ is irradiated, versus other sites that are either bilateral or where only part of the organ is treated, such as the skin and lung. This study supports the use of local therapies to treat stage IE and IIE MZL. 

Keyword(s): Marginal zone, Radiation

Session topic: Treatment and outcome in non-Hodgkin lymphomas
Abstract: S108

Type: Oral Presentation

Presentation during EHA20: From 12.06.2015 12:00 to 12.06.2015 12:15

Location: Room A7

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, infrequently, 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.

Aims
To study patient characteristics and long-term outcome of a large cohort of patients with early-stage localized extra-nodal marginal zone lymphoma

Methods
We identified 490 consecutive patients with stage IE or IIE MZL referred between 1992 and 2012 to Memorial Sloan Kettering Cancer Center. Pathology was confirmed by hematopathologists at our institution. Patient and disease factors as well as treatment types were analyzed for association with relapse-free survival (RFS), overall survival (OS), and cumulative incidence of relapse.

Results

Median follow-up was 4.8 years. Median patient age was 60 years and 57% were female. Ann-Arbor stage was IE in 89%. Most common sites were stomach (32%), orbit (14%), lung (12%), skin (12%), and parotid (5%). Radiotherapy alone (RT) was the initial treatment in 50% of patients, followed by surgical resection (30%), observation (9%), immunotherapy (4%), and chemotherapy (2%).

Five- year OS and RFS were 90% and 64%, respectively; 10-year OS and RFS were 73% and 45%. Disease-specific death was 1.3% at 5 years and 1.8% at 10 years. Cumulative incidence of progression/relapse was 29% by 5 years and 39% by 10 years. Amongst the 384 patients with complete response (CR), 99 patients experienced relapse. On multivariable analysis, initial treatment type and primary disease site were independently associated with RFS and relapse (all p<0.005). All disease sites (HR>2.0, p≤0.01) except for thyroid (p=0.8) had worse RFS relative to stomach.  Compared with RT, chemotherapy or immunotherapy had worse RFS (HR 2.2, p=0.004) while surgery was no different (p=0.52). After RT, only 11 patients experienced in-field failure, with a 5-year cumulative incidence of 2.4%. Most common location of relapse after CR was distant; relapses were also observed in paired untreated organs, such as the orbit, salivary gland, and breast. Crude rate of transformation to pathologically-confirmed large-cell lymphoma was 2% (11 patients). Second tumors in irradiated sites developed in 3 patients: 2 of these were breast ductal carcinoma in situ cured with surgical resection.



Summary
Overall and cause-specific survival are excellent in early-stage extra-nodal MZL. Treatment with RT or surgery was associated with longer RFS and reduced the need for salvage. Relapses are common after initial remission, and most frequently occur in distant sites. Transformation to large cell lymphomas is rare. Stomach cases are less likely to relapse than other anatomic primary sites, perhaps in part because the entire organ is irradiated, versus other sites that are either bilateral or where only part of the organ is treated, such as the skin and lung. This study supports the use of local therapies to treat stage IE and IIE MZL. 

Keyword(s): Marginal zone, Radiation

Session topic: Treatment and outcome in non-Hodgkin lymphomas

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