title: Extranodal Marginal Zone B-cell Lymphoma of the Ocular Adnexa.
abstract: BACKGROUND
  Low-grade B-cell lymphomas located around the eye present unique challenges in diagnosis and treatment. Extranodal marginal zone B-cell lymphoma is the most common lymphoma of the ocular adnexa (conjunctiva, orbit, lacrimal gland, and eyelid).
  
  
  METHODS
  A systematic search of the relevant literature was performed. Material pertinent to the diagnosis, prognosis, pathogenesis, and treatment of extranodal marginal zone B-cell lymphoma of the ocular adnexa was identified, reviewed, and analyzed, focusing on management strategies for primary localized disease.
  
  
  RESULTS
  The primary cause of extranodal marginal zone B-cell lymphoma of the ocular adnexa remains elusive, although an infectious agent is suspected. Radiotherapy is the most common initial treatment for localized disease. Initial treatment with chemotherapy, immunotherapy, and antibiotics has shown promising results, but the number of series is limited and controlled trials do not exist.
  
  
  CONCLUSIONS
  Although the long-term outcome of localized extranodal marginal zone B-cell lymphoma of the ocular adnexa is good, optimal treatment remains a goal. The variation in rates of local and systemic relapse among treated stage 1E tumors suggests that critical factors affecting outcomes are not fully understood. Radiotherapy is the standard of care; at this time, the evidence is insufficient to recommend chemotherapy, immunotherapy, or antibiotics for initial treatment of extranodal marginal zone B-cell lymphoma localized to the ocular adnexa. Well-controlled comparative studies are needed.
authors.affiliation: Department of Pathology and Cell Biology, Department of Ophthalmology, University of South Florida Morsani College of Medicine, Tampa 33612, USA. cmargo@health.usf.edu.
authors.forename: Jean
authors.initials: J
authors.lastname: Guffey Johnson
authors.forename: Lauren A
authors.initials: LA
authors.lastname: Terpak
authors.forename: Curtis E
authors.initials: CE
authors.lastname: Margo
authors.forename: Reza
authors.initials: R
authors.lastname: Setoodeh
mesh_terms: D005134:Eye Neoplasms / Q000188:drug therapy* / Q000628:therapy*; D006801:Humans; D018442:Lymphoma, B-Cell, Marginal Zone / Q000188:drug therapy* / Q000628:therapy*; D011379:Prognosis