T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases.
OBJECTIVE
Lymphomas of the eye and its adnexa are frequently of B lineage. This study aims to characterize the clinical and histopathologic features of the rare non-B-cell non-Hodgkin lymphomas (NHL) of these locations.


METHODS
Retrospective, noncomparative case series.


METHODS
Seven cases of T- and T/NK-cell lymphomas involving the ocular and ocular adnexal tissues.


METHODS
A morphologic, immunohistochemical, and molecular analysis (polymerase chain reaction) of each of the tumors was undertaken. The lesions were classified according to the Revised European-American Lymphoma (REAL) classification. The clinical and follow-up data were collected.


RESULTS
The patients included four women and three men ranging in age from 32 to 88 years (mean, 63 years). The presenting ophthalmic symptoms varied from a small nodule on the upper eyelid and conjunctival swellings to dramatic loss of vision associated with gross protrusion of the globe. Five of the cases presented were secondary manifestations of a systemic lymphoma in ocular tissues; two cases represented primary disease. Three cases were "peripheral T-cell lymphomas (PTCL), unspecified" with positivity for CD3, CD8, and betaF1 and negativity for CD56. Two cases were CD3+, CD30+, and CD56- and were classified as "anaplastic large-cell lymphomas of T-cell type" (T-ALCL). The remaining two cases showed an immunophenotype of CD3+, CD56+, and betaF1- and proved to contain Epstein-Barr virus (EBV) by in situ hybridization, consistent with "T/natural killer (NK)-cell lymphoma of nasal type." Clonal T-cell populations were shown in all three of the PTCLs by Southern blot (n = 1) and polymerase chain reaction (n = 2) for the T-cell receptor gamma and beta genes in one case of ALCL but not in the T/NK-cell lymphomas. Five patients died within 2 years; only two patients (one primary PTCL and one cutaneous T-ALCL) were disease free at 4 and 5 years' follow-up, respectively.


CONCLUSIONS
This study shows that a heterogeneous group of T-cell lymphomas can involve the eye and its adnexal tissue. Most T-cell neoplasms are secondary manifestations of systemic disease and carry a poor prognosis. These findings, in conjunction with published data on ocular B-NHL, also indicate that immunophenotypic differentiation between T- and B-NHL in these locations is of clinical importance.
Coupland
S E
SE
Department of Pathology, University Hospital Benjamin Franklin, Berlin, Germany. dsh@pathpaul.ukbf.fu-berlin.de
Foss
H D
HD
Assaf
C
C
Auw-Haedrich
C
C
Anastassiou
G
G
Anagnostopoulos
I
I
Hummel
M
M
Karesh
J W
JW
Lee
W R
WR
Stein
H
H
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D015703:Antigens, CD / Q000032:analysis; D020031:Epstein-Barr Virus Infections / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D005134:Eye Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000821:virology; D005260:Female; D015333:Gene Rearrangement, beta-Chain T-Cell Antigen Receptor / Q000235:genetics; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D006801:Humans; D016130:Immunophenotyping; D017403:In Situ Hybridization; D007694:Killer Cells, Natural / Q000737:chemistry / Q000473:pathology*; D017728:Lymphoma, Large-Cell, Anaplastic / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000821:virology; D008228:Lymphoma, Non-Hodgkin / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000821:virology; D016399:Lymphoma, T-Cell / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000821:virology; D016410:Lymphoma, T-Cell, Cutaneous / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000821:virology; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics / Q000276:immunology / Q000473:pathology / Q000821:virology; D008297:Male; D008875:Middle Aged; D016133:Polymerase Chain Reaction; D012367:RNA, Viral / Q000032:analysis; D012189:Retrospective Studies; D012878:Skin Neoplasms / Q000235:genetics / Q000276:immunology / Q000473:pathology* / Q000821:virology; D013601:T-Lymphocytes / Q000737:chemistry / Q000473:pathology*
D015703:Antigens, CD; D012367:RNA, Viral