Lymphoproliferative lesions of the ocular adnexa. Analysis of 112 cases.
OBJECTIVE
Lymphoproliferative lesions of the ocular adnexa were analyzed to examine (1) the suitability of the Revised European-American Lymphoma (REAL) classification for the subtyping of the lymphomas in these sites; (2) the predictive value of the REAL classification for the evolution of these tumors; and (3) the frequency and prognostic impact of tumor type, location, proliferation rate (Ki-67 index), p53, CD5 positivity and the presence of monoclonality within these tumors.


METHODS
Retrospective review.


METHODS
The clinical, histomorphologic, immunohistochemical, and molecular biologic (polymerase chain reaction [PCR]) features of lymphoid proliferations of the ocular adnexa were studied. STUDY MATERIALS: The ocular adnexal lymphoproliferative lesions were located as follows: orbit in 52 patients (46%), conjunctiva in 32 patients (29%), eyelid in 23 patients (21%), and caruncle in 5 patients (4%).


RESULTS
Reactive lymphoid hyperplasia was diagnosed in 12 cases and lymphoma in 99 cases; 1 case remained indeterminate. The five main subtypes of lymphoma according to the REAL classification were extranodal marginal-zone B-cell lymphoma (64%), follicle center lymphoma (10%), diffuse large cell B-cell lymphoma (9%), plasmacytoma (6%), and lymphoplasmocytic lymphoma (5%). Age, gender, and anatomic localization of the lymphomas did not have prognostic significance during a follow-up period of 6 months to 16.5 years (mean, 3.3 years). Extent of disease at time of presentation was the most important clinical prognostic factor: advanced disease correlated with increased risk ratios of having persistent disease at the final follow-up and with lymphoma-related death (P < 0.001). Histomorphologic features and immunohistochemical markers positively correlating with disseminated disease at presentation, stage at final follow-up, and occurrence of lymphoma-related death included cytologic atypia (P < 0.001), MIB-1 proliferation rate (P < 0.001), and tumor cell p53 positivity (P < 0.001). The MIB-1 proliferation rates greater than 20% in extranodal marginal-zone B-cell lymphoma corresponded to at least stage II lymphoma (P < 0.05).


CONCLUSIONS
The REAL classification is suitable for the subdivision of the ocular adnexal lymphomas. The MIB-1 proliferation rate and p53 positivity may aid the prediction of disease stage and disease progression, whereas PCR can support the diagnosis and reduce the number of histologically indeterminate lesions.
Coupland
S E
SE
Department of Pathology, Universitätsklinikum Benjamin Franklin, Freie Universität, Berlin, Germany.
Krause
L
L
Delecluse
H J
HJ
Anagnostopoulos
I
I
Foss
H D
HD
Hummel
M
M
Bornfeld
N
N
Lee
W R
WR
Stein
H
H
D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000378:metabolism; D003230:Conjunctival Neoplasms / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D004273:DNA, Neoplasm / Q000032:analysis; D005142:Eyelid Neoplasms / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D019394:Ki-67 Antigen / Q000378:metabolism; D007766:Lacrimal Apparatus Diseases / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D008223:Lymphoma / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D008940:Mitotic Index; D009918:Orbital Neoplasms / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D016133:Polymerase Chain Reaction; D011379:Prognosis; D019310:Pseudolymphoma / Q000145:classification / Q000378:metabolism / Q000473:pathology*; D012189:Retrospective Studies; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism
D015703:Antigens, CD; D004273:DNA, Neoplasm; D019394:Ki-67 Antigen; D016159:Tumor Suppressor Protein p53