title: Ocular adnexal lymphoproliferative disease: a series of 73 cases.
abstract: BACKGROUND
  This study involved 73 patients with lymphoproliferative lesions of the ocular adnexa. The lesions were categorized using the Revised European American Lymphoma classification of lymphoid tissues and analysed to determine the frequency and prognostic impact of tumour type, location, stage and patient's age and sex.
  
  
  METHODS
  The clinical, histopathological, immunohistochemical and phenotypic analysis by flow cytometry and follow-up data were studied.
  
  
  RESULTS
  The ocular adnexal lymphoproliferative lesions included 70 lymphomas and six reactive lymphoid hyperplasia. Seventy-nine per cent had stage IE disease, 4% stage II, 1.5% stage III and 15.5% stage IV. Five patients (7%) had a past history of systemic lymphoma. Major histological types were extranodal marginal zone lymphoma (MZL) in 44 (63%), follicular (FL) in 12 (17%), diffuse large B-cell (DLBCL) in eight (11%), mantle cell (MCL) in two (3%), B-cell chronic lymphocytic leukaemia (CLL)/small lymphocytic lymphoma in two (3%), peripheral T-cell lymphoma (PTCL) one (1.5%) and natural killer cell lymphoma (NKCL) in one (1.5%). Longest survival was seen in those with low-grade lymphomas (MZL and FL) and worst in PTCL and NKCL. Lymphoma-related mortality was 2% for MZL, 33% for FL, 38% for DLBCL, and 100% for MCL, PTCL and NKCL. Systemic lymphoma was present prior to, at presentation or at subsequent follow up in 26/68 (39%) of all lymphoma patients, 17% for MZL, 38% for DLBCL, 83% for FL, and 100% for MCL, CLL, PTCL and NKCL.
  
  
  CONCLUSIONS
  The majority of ocular adnexal lymphomas were low-grade B-cell lymphomas (MZL). Multivariate analysis showed that the only significant independent predictors of all causes of mortality were the histological type of lymphoma and the stage of disease at presentation.
authors.affiliation: Department of Anatomical Pathology, St Vincent's Hospital, Fitzroy, Victoria, Australia. mckelvpa@svhm.org.au
authors.forename: P A
authors.initials: PA
authors.lastname: McKelvie
authors.forename: A
authors.initials: A
authors.lastname: McNab
authors.forename: I C
authors.initials: IC
authors.lastname: Francis
authors.forename: R
authors.initials: R
authors.lastname: Fox
authors.forename: J
authors.initials: J
authors.lastname: O'Day
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D003230:Conjunctival Neoplasms / Q000145:classification / Q000401:mortality / Q000473:pathology*; D005142:Eyelid Neoplasms / Q000145:classification / Q000401:mortality / Q000473:pathology*; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D016130:Immunophenotyping; D008223:Lymphoma / Q000145:classification / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D009918:Orbital Neoplasms / Q000145:classification / Q000401:mortality / Q000473:pathology*; D011379:Prognosis; D015996:Survival Rate