TY - JOUR
T1 - Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the salivary glands
T2 - A multicenter, international experience of 248 patients (IELSG 41)
AU - Jackson, Amie E.
AU - Mian, Michael
AU - Kalpadakis, Christina
AU - Pangalis, Gerassimos A.
AU - Stathis, Anastasios
AU - Porro, Elena
AU - Conconi, Annarita
AU - Cortelazzo, Sergio
AU - Gaidano, Gianluca
AU - Guillermo, Armando Lopez
AU - Johnson, Peter W.
AU - Martelli, Maurizio
AU - Martinelli, Giovanni
AU - Thieblemont, Catherine
AU - McPhail, Ellen D.
AU - Copie-Bergman, Christiane
AU - Pileri, Stefano A.
AU - Jack, Andrew
AU - Campo, Elias
AU - Mazzucchelli, Luca
AU - Ristow, Kay
AU - Habermann, Thomas M.
AU - Cavalli, Franco
AU - Nowakowski, Grzegorz S.
AU - Zucca, Emanuele
N1 - Publisher Copyright:
© AlphaMed Press 2015.
PY - 2015/8/12
Y1 - 2015/8/12
N2 - Background. The salivary gland is one of the most common sites involved by nongastric, extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT). A large series of patients with long-term follow-up has not been documented. This multicenter, international study sought to characterize the clinical characteristics, treatment, and natural history of salivary gland MALT lymphoma. Methods. Patients with biopsy-confirmed salivary gland MALT lymphoma were identified from multiple international sites. Risk factors, treatment, and long-term outcomes were evaluated. Results. A total of 247 patients were evaluated; 76% presented with limited-stage disease. There was a history of autoimmune disorder in 41%, with Sjögren disease being the most common (83%). Fifty-seven percent of patients were initially treated with local therapy with surgery, radiation, or both; 37 of patients were treated with systemic therapy initially, with 47% of those receiving rituximab; and 6% of patients were observed. The median overall survival (OS) was 18.3 years. The median progression-free survival (PFS) following primary therapy was 9.3 years. There was no difference in the outcomes between patients receiving local or systemic therapy in first-line management. On multivariate analysis, age <60 years and low to intermediate international prognostic index were associated with improved OS and PFS; Sjögren disease was associated with improved OS. Conclusion. Salivary gland MALT lymphoma has an excellent prognosis regardless of initial treatment, and patients with Sjögren disease have improved survival. Risks for long-term complications must be weighed when determining initial therapy.
AB - Background. The salivary gland is one of the most common sites involved by nongastric, extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue (MALT). A large series of patients with long-term follow-up has not been documented. This multicenter, international study sought to characterize the clinical characteristics, treatment, and natural history of salivary gland MALT lymphoma. Methods. Patients with biopsy-confirmed salivary gland MALT lymphoma were identified from multiple international sites. Risk factors, treatment, and long-term outcomes were evaluated. Results. A total of 247 patients were evaluated; 76% presented with limited-stage disease. There was a history of autoimmune disorder in 41%, with Sjögren disease being the most common (83%). Fifty-seven percent of patients were initially treated with local therapy with surgery, radiation, or both; 37 of patients were treated with systemic therapy initially, with 47% of those receiving rituximab; and 6% of patients were observed. The median overall survival (OS) was 18.3 years. The median progression-free survival (PFS) following primary therapy was 9.3 years. There was no difference in the outcomes between patients receiving local or systemic therapy in first-line management. On multivariate analysis, age <60 years and low to intermediate international prognostic index were associated with improved OS and PFS; Sjögren disease was associated with improved OS. Conclusion. Salivary gland MALT lymphoma has an excellent prognosis regardless of initial treatment, and patients with Sjögren disease have improved survival. Risks for long-term complications must be weighed when determining initial therapy.
KW - B-cell
KW - Lymphoid tissue
KW - Lymphoma
KW - Marginal zone
KW - Salivary glands
KW - Sjögren’s disease
UR - http://www.scopus.com/inward/record.url?scp=84943276711&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=84943276711&partnerID=8YFLogxK
U2 - 10.1634/theoncologist.2015-0180
DO - 10.1634/theoncologist.2015-0180
M3 - Article
C2 - 26268740
AN - SCOPUS:84943276711
SN - 1083-7159
VL - 20
SP - 1149
EP - 1153
JO - Oncologist
JF - Oncologist
IS - 10
ER -