Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical and cytogenetical characteristics
Lene Dissing Sjo 1,
Steffen Heegaard 2*,
Jan Ulrik Prause 1,
Bodil Laub Petersen 3,
Sanni Pedersen 3,
and
Elisabeth Ralfkiaer 3
1 Dept. of Neuroscience and Pharmacology, Eye Pathology Institute, University of Copenhagen, Copenhagen, Denmark
2 University of Copenhagen, Eye Pathology Institute, Frederik V's Vej 11, Copenhagen, DK-2100, Denmark
3 Copenhagen University Hospital, Rigshospitalet, Dept. of Pathology, Copenhagen, Denmark
* To whom correspondence should be addressed. E-mail: sthe{at}sund.ku.dk.
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Abstract |
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PURPOSE. To evaluate clinical-, immunophenotypical- and cytogenetical characteristics of 116 patients with a diagnosis of extranodal marginal zone lymphoma (EMZL) presenting primarily in the ocular region.
METHODS. Specimens from all patients diagnosed with ophthalmic lymphoma in Denmark during the period 1980 to 2005 were reviewed and reclassified according to the WHO classification. Cases reclassified as EMZL were selected and reviewed with respect to clinical characteristics and outcome. The presence of translocations involving IGH and/or MALT1 was investigated in 42 specimens by fluorescence in situ hybridization (FISH).
RESULTS. Median age was 69 years. Most lymphomas were located in the orbit. Around one fourth of patients had disseminated disease at presentation. One third of patients had a relapse or progression of disease after initial therapy and relapses were frequently found at extra-ocular sites. Five-year progression free survival and overall survival rates were 71% and 75% respectively. Translocations involving the IGH- or MALT1gene loci were detected in two of 42 specimens (5%). In Cox regression multivariate analysis IGH-translocation was the only factor associated with PFS whereas favorable International Prognostic Index (IPI) score was the most reliable predictor of OS.
CONCLUSIONS. EMZL presenting in the ocular region usually runs an indolent course, but relapses are frequently seen. The IPI-score was the most reliable independent parameter for estimating risk of death in our cohort of patients. Furthermore, we found that the frequency of translocations involving the MALT1- and IGH-gene loci is low in ocular region EMZL.
Key Words:
immunopathology, in situ hybridization, fluorescent probes
