Br J Ophthalmol 2000;84:907-913
( August )
Histological features of ocular adnexal lymphoma (REAL
classification) and their association with patient morbidity and
survival
Christopher Jenkinsa, Geoffrey E Rosea, Catey Bunceb, John E Wrighta, Ian A Creec, Nicholas Plowmand, Sue Lightmane, Ivan Moseleya, Andrew Nortonf
a Orbital Clinic,
Moorfields Eye Hospital, b Glaxo Department of
Epidemiology, Moorfields Eye Hospital, c Department of Pathology, Institute of
Ophthalmology, d Department
of Radiotherapy, St Bartholomew's Hospital, e Department of Clinical Ophthalmology, Moorfields
Eye Hospital, f Department of
Pathology, St Bartholomew's Hospital
Correspondence to: Mr Geoffrey E Rose, Orbital Clinic,
Moorfields Eye Hospital, City Road, London EC1V 2PD
Accepted for publication 16 March 2000
BACKGROUND The
histological characteristics of ocular adnexal lymphomas have
previously provided only a limited guide to clinical outcome for
affected patients. This clinicopathological relation was re-examined using the Revised European American Lymphoma (REAL) system to classify
the tumours in a large cohort of patients.
METHODSThe biopsies
and clinical follow up data for 192 patients with ocular adnexal
lymphoma were reviewed, the biopsies being regraded in accordance with
the REAL classification. For each of five histological groups, logistic
regression analysis was used to determine the odds ratios (OR) for the
presence of systemic disease at the time of orbital diagnosis and Cox
regression analysis was used to assess the hazard ratios (HR) for
disseminated disease and lymphoma related death. For 108 patients in
whom extraorbital spread occurred, the histological category of
lymphoma was compared with the sites of dissemination.
RESULTSAt
presentation, the frequency of previous or concurrent extraorbital
disease increased from marginal zone lymphoma (OR 1.0), diffuse
lymphoplasmacytic/lymphoplasmacytoid lymphoma (OR 2.3), follicle
centre lymphoma (OR 3.8), diffuse large B cell lymphoma (OR 4.0) to
other histological lymphoma variants (OR 26.8). For all histological
types, the estimated risk of extraorbital disease and lymphoma related
death continued for many years and the proportion of patients with at
least one extraorbital recurrence after 5 years was 47% for MZL, 48%
for LPL, 64% for FCL, 81% for DLCL, and 95% for other lymphoma
variants. The corresponding estimated rates for 5 year lymphoma related
mortality were 12%, 19%, 22%, 48%, and 53% respectively.
CONCLUSIONSPatients
with ocular adnexal lymphoma can be classified by REAL into five
distinct groups, which show a progressive increase in the risks of
extraorbital disease at diagnosis, of disease dissemination with time,
and of tumour related death.
© 2000 by British Journal of Ophthalmology
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