title: Clinical features, treatment and outcome of mucosa-associated lymphoid tissue (MALT) lymphoma of the ocular adnexa: single center experience of 60 patients.
abstract: BACKGROUND
  Orbital marginal zone B-cell lymphoma (OAML) constitutes for the most frequent diagnosis in orbital lymphoma. Relatively little data, however, have been reported in larger cohorts of patients staged in a uniform way and no therapy standard exists to date.
  
  
  METHODS
  We have retrospectively analyzed 60 patients diagnosed and treated at our institution 1999-2012. Median age at diagnosis was 64 years (IQR 51-75) and follow-up time 43 months (IQR 16-92). All patients had undergone uniform extensive staging and histological diagnosis was made by a reference pathologist according to the WHO classification.
  
  
  RESULTS
  The majority of patients presented with stage IE (n = 40/60, 67%), three had IIE/IIIE and the remaining 17 stage IVE. Seven patients with IVE had bilateral orbital disease whereas the others showed involvement of further organs. Treatment data were available in 58 patients. Local treatment with radiotherapy (14/58, 24%) or surgery (3/58, 5%) resulted in response in 82% of patients. A total of 26 patients (45%) received systemic treatment with a response rate of 85%. Nine patients received antibiotics as initial therapy; response rate was 38%. Watchful-waiting was the initial approach in 6/58 patients. In total 28/58 patients (48%) progressed and were given further therapy. Median time-to-progression in this cohort was 20 months (IQR 9-39). There was no difference in time-to-progression after first-line therapy between the different therapy arms (p = 0.14). Elevated beta-2-microglobulin, plasmacytic differentiation, autoimmune disorder and site of lymphoma were not associated with a higher risk for progress.
  
  
  CONCLUSIONS
  Our data underscore the excellent prognosis of OAML irrespective of initial therapy, as there was no significant difference in time-to-progression and response between local or systemic therapy. In the absence of randomized trials, the least toxic individual approach should be chosen for OAML.
authors: Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria.
authors: Barbara
authors: B
authors: Kiesewetter
authors: Department of Ophthalmology, Medical University of Vienna, Vienna, Austria.
authors: Julius
authors: J
authors: Lukas
authors: Department of Ophthalmology, Medical University of Vienna, Vienna, Austria.
authors: Andreas
authors: A
authors: Kuchar
authors: Department of Radiology, Medical University of Vienna, Vienna, Austria.
authors: Marius E
authors: ME
authors: Mayerhoefer
authors: Department of Pathology, Medical University of Vienna, Vienna, Austria.
authors: Berthold
authors: B
authors: Streubel
authors: Department of Medicine I, Division of Infectious Diseases and Tropical Medicine, Medical University of Vienna, Vienna, Austria.
authors: Heimo
authors: H
authors: Lagler
authors: Department of Pathology, Medical University of Vienna, Vienna, Austria.
authors: Leonhard
authors: L
authors: Müllauer
authors: Department of Medicine I, Division of Bone Marrow Transplantation, Medical University of Vienna, Vienna, Austria.
authors: Stefan
authors: S
authors: Wöhrer
authors: Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria.
authors: Julia
authors: J
authors: Fischbach
authors: Department of Medicine I, Division of Oncology, Medical University of Vienna, Vienna, Austria.
authors: Markus
authors: M
authors: Raderer
mesh_terms: D000368:Aged; D003131:Combined Modality Therapy; D018450:Disease Progression; D005260:Female; D006801:Humans; D053208:Kaplan-Meier Estimate; 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; D016896:Treatment Outcome