title: Primary mucosa-associated lymphoid tissue lymphoma of the salivary glands: a multicenter Rare Cancer Network study.
abstract: OBJECTIVE
  Involvement of salivary glands with mucosa-associated lymphoid tissue (MALT) lymphoma is rare. This retrospective study was performed to assess the clinical profile, treatment outcome, and prognostic factors of MALT lymphoma of the salivary glands.
  
  
  METHODS
  Thirteen member centers of the Rare Cancer Network from 10 countries participated, providing data on 63 patients. The median age was 58 years; 47 patients were female and 16 were male. The parotid glands were involved in 49 cases, submandibular in 15, and minor glands in 3. Multiple glands were involved in 9 patients. Staging was as follows: IE in 34, IIE in 12, IIIE in 2, and IV in 15 patients.
  
  
  RESULTS
  Surgery (S) alone was performed in 9, radiotherapy (RT) alone in 8, and chemotherapy (CT) alone in 4 patients. Forty-one patients received combined modality treatment (S + RT in 23, S + CT in 8, RT + CT in 4, and all three modalities in 6 patients). No active treatment was given in one case. After initial treatment there was no tumor in 57 patients and residual tumor in 5. Tumor progression was observed in 23 (36.5%) (local in 1, other salivary glands in 10, lymph nodes in 11, and elsewhere in 6). Five patients died of disease progression and the other 5 of other causes. The 5-year disease-free survival, disease-specific survival, and overall survival were 54.4%, 93.2%, and 81.7%, respectively. Factors influencing disease-free survival were use of RT, stage, and residual tumor (p < 0.01). Factors influencing disease-specific survival were stage, recurrence, and residual tumor (p < 0.01).
  
  
  CONCLUSIONS
  To our knowledge, this report represents the largest series of MALT lymphomas of the salivary glands published to date. This disease may involve all salivary glands either initially or subsequently in 30% of patients. Recurrences may occur in up to 35% of patients at 5 years; however, survival is not affected. Radiotherapy is the only treatment modality that improves disease-free survival.
authors.affiliation: Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey. yavuz.anacak@ege.edu.tr
authors.forename: Yavuz
authors.initials: Y
authors.lastname: Anacak
authors.forename: Robert C
authors.initials: RC
authors.lastname: Miller
authors.forename: Nikos
authors.initials: N
authors.lastname: Constantinou
authors.forename: Angela M
authors.initials: AM
authors.lastname: Mamusa
authors.forename: Ron
authors.initials: R
authors.lastname: Epelbaum
authors.forename: Yexiong
authors.initials: Y
authors.lastname: Li
authors.forename: Anna Lucas
authors.initials: AL
authors.lastname: Calduch
authors.forename: Anna
authors.initials: A
authors.lastname: Kowalczyk
authors.forename: Damien C
authors.initials: DC
authors.lastname: Weber
authors.forename: Sidney P
authors.initials: SP
authors.lastname: Kadish
authors.forename: Nuran
authors.initials: N
authors.lastname: Bese
authors.forename: Philip
authors.initials: P
authors.lastname: Poortmans
authors.forename: Serra
authors.initials: S
authors.lastname: Kamer
authors.forename: Mahmut
authors.initials: M
authors.lastname: Ozsahin
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D003131:Combined Modality Therapy / Q000379:methods; D018450:Disease Progression; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D008297:Male; D008875:Middle Aged; D009367:Neoplasm Staging; D010307:Parotid Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy; D035583:Rare Diseases / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D012189:Retrospective Studies; D012468:Salivary Gland Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D012470:Salivary Glands, Minor; D013365:Submandibular Gland Neoplasms / Q000401:mortality / Q000473:pathology / Q000628:therapy