Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue of the salivary glands: A multicenter, international experience of 248 patients (IELSG 41)

Amie E. Jackson, Michael Mian, Christina Kalpadakis, Gerassimos A. Pangalis, Anastasios Stathis, Elena Porro, Annarita Conconi, Sergio Cortelazzo, Gianluca Gaidano, Armando Lopez Guillermo, Peter W. Johnson, Maurizio Martelli, Giovanni Martinelli, Catherine Thieblemont, Ellen D. McPhail, Christiane Copie-Bergman, Stefano A. Pileri, Andrew Jack, Elias Campo, Luca MazzucchelliKay Ristow, Thomas M. Habermann, Franco Cavalli, Grzegorz S. Nowakowski, Emanuele Zucca

Research output: Contribution to journalArticlepeer-review

26 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)1149-1153
Number of pages5
Issue number10
StatePublished - Aug 12 2015


  • B-cell
  • Lymphoid tissue
  • Lymphoma
  • Marginal zone
  • Salivary glands
  • Sjögren’s disease

ASJC Scopus subject areas

  • Oncology
  • Cancer Research


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