Extranodal marginal zone lymphoma in the ocular region: clinical, immunophenotypical, and cytogenetical characteristics.
OBJECTIVE
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 with a diagnosis of ophthalmic lymphoma in Denmark during the period 1980 to 2005 were reviewed and reclassified according to the World Health Organization (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. Approximately one fourth of the patients had disseminated disease at presentation. One third experienced a relapse or progression of disease after initial therapy, and relapses were frequently found at extraocular sites. Five-year progression-free survival and overall survival (OS) rates were 71% and 75%, respectively. Translocations involving the IGH- or MALT1-gene loci were detected in 2 (5%) of 42 specimens. In Cox regression multivariate analysis, IGH-translocation was the only factor associated with PFS, whereas a 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.
Sjö
Lene D
LD
Eye Pathology Institute, Department of Neuroscience and Pharmacology, University of Copenhagen, Copenhagen, Denmark.
Heegaard
Steffen
S
Prause
Jan U
JU
Petersen
Bodil L
BL
Pedersen
Sanni
S
Ralfkiaer
Elisabeth
E
D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000970:Antineoplastic Agents / Q000627:therapeutic use; D000074018:B-Cell CLL-Lymphoma 10 Protein; D020169:Caspases / Q000235:genetics; D002648:Child; D003582:Cytogenetics; D005134:Eye Neoplasms / Q000235:genetics* / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D005260:Female; D050438:Genes, Immunoglobulin Heavy Chain / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D017404:In Situ Hybridization, Fluorescence; D018442:Lymphoma, B-Cell, Marginal Zone / Q000235:genetics* / Q000401:mortality / Q000473:pathology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D000074884:Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein; D009363:Neoplasm Proteins / Q000235:genetics; D011379:Prognosis; D011878:Radiotherapy; D015996:Survival Rate; D014178:Translocation, Genetic; D055815:Young Adult
D048868:Adaptor Proteins, Signal Transducing; D000970:Antineoplastic Agents; D000074018:B-Cell CLL-Lymphoma 10 Protein; C118591:BCL10 protein, human; D009363:Neoplasm Proteins; D020169:Caspases; C400109:MALT1 protein, human; D000074884:Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein