title: Primary ocular adnexal MALT lymphoma: a long-term follow-up study of 114 patients.
abstract: BACKGROUND
  Although primary ocular adnexal MALT (mucosa-associated lymphoid tissue) lymphoma (POAML) is a recently recognized unique entity, its natural history, prognostic factors, behavior of progression and death, and standard initial management have not been fully elucidated.
  
  
  METHODS
  The data of 114 patients with histologically verified POAML who were treated at our institution between 1970 and 2003 were retrospectively analyzed.
  
  
  RESULTS
  With a median follow-up duration of 5.7 years (0.6-34.0), estimated overall survival (OS) rate and progression-free survival (PFS) rate at 10 years was 89% and 57%, respectively. Thirteen (11%) patients died, but only three (3%) of them due to progressive lymphoma. Thirty-one (27%) patients progressed: eight who progressed at contra lateral sites were limited to those who had initially involved in the orbit (P = 0.036) and their OS and PFS were significantly longer (P = 0.035 and 0.039, respectively). Patients who initially received radiation-containing therapy were superior in PFS but not in OS to those initially treated with other modalities (P = 0.016 and 0.091, respectively). When we compared the outcomes of the observation cohort and the immediate therapy cohort, there were no significant differences in OS and PFS (P = 0.499 and 0.073, respectively).
  
  
  CONCLUSIONS
  The majority of patients with POAML showed the behaviors of localized and indolent diseases. Our preliminary observation that no initial therapy is an acceptable approach for selected patients was confirmed. Considering the possible heterogeneity of POAML among initial sites, further investigations are warranted.
authors.affiliation: Hematology and Stem Cell Transplantation Division, National Cancer Center Hospital and Research Institute, Tokyo, Japan.
authors.forename: Kazuki
authors.initials: K
authors.lastname: Tanimoto
authors.forename: Akihiro
authors.initials: A
authors.lastname: Kaneko
authors.forename: Shigenobu
authors.initials: S
authors.lastname: Suzuki
authors.forename: Naohiro
authors.initials: N
authors.lastname: Sekiguchi
authors.forename: Takashi
authors.initials: T
authors.lastname: Watanabe
authors.forename: Yukio
authors.initials: Y
authors.lastname: Kobayashi
authors.forename: Yoshikazu
authors.initials: Y
authors.lastname: Kagami
authors.forename: Akiko Miyagi
authors.initials: AM
authors.lastname: Maeshima
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Matsuno
authors.forename: Kensei
authors.initials: K
authors.lastname: Tobinai
mesh_terms: D018450:Disease Progression; D005134:Eye Neoplasms / Q000401:mortality* / Q000628:therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000401:mortality* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000401:mortality / Q000628:therapy; D012189:Retrospective Studies; D015996:Survival Rate