Elsevier

Ophthalmology

Volume 106, Issue 11, 1 November 1999, Pages 2109-2120
Ophthalmology

T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: A clinicopathologic, immunohistochemical, and molecular study of seven cases

https://doi.org/10.1016/S0161-6420(99)90492-XGet rights and content

Abstract

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.

Design

Retrospective, noncomparative case series.

Participants

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 βF1 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 βF1− 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.

Conclusion

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.

Section snippets

Case histories

A summary of the relevant clinical information is presented in Table 1.

Tissue samples

Collections on file in the Departments of Pathology, Western Infirmary, Glasgow, Scotland, and the University Hospital Benjamin Franklin, Berlin, Germany, as well as those in the Departments of Ophthalmology, University Hospital Freiburg, Germany, and the University of Maryland School of Medicine, Baltimore, Maryland, were reviewed. Cases of mycosis fungoides were excluded. Most tissue biopsy specimens had been fixed in 4% formaldehyde solution and embedded in paraffin; some biopsy specimens

Results

The results of the immunohistochemical, in situ hybridization, and molecular biologic findings of the cases examined are summarized in Table 2.

Discussion

Lymphomas with T-cell phenotype are uncommon, accounting for only 10% to 15% of all NHL.27 They represent a heterogeneous group of diseases differing in histopathologic analysis, tumor site, and cell origin. They include not only PTCLs derived from α/β cells but also some recently recognized entities such as T/NK-cell lymphomas and γ/δ-hepatosplenic lymphomas.5 The T-cell lymphomas are rare in ocular tissues and are represented predominantly by mycosis fungoides and secondary manifestations of

Acknowledgements

The authors thank Dr. A. Hidayat, who was involved in the diagnosis of case 6; Dr. A. Kapp for his assistance in obtaining clinical information; as well as Mr. H-H. Müller, Mrs. H. Protz, and Miss D. Jahnke for their supportive technical assistance.

References (57)

  • T. Kohno et al.

    Ocular manifestations of adult T-cell leukemia/lymphoma

    A clinicopathologic study. Ophthalmology

    (1993)
  • T. Uchiyama et al.

    Adult T-cell leukemiaclinical and hematologic features of 16 cases

    Blood

    (1977)
  • S.M. Whitcup et al.

    Intraocular lymphoma. Clinical and histopathologic diagnosis

    Ophthalmology

    (1993)
  • D.M. Knowles et al.

    Lymphoid hyperplasia and malignant lymphoma occurring in the ocular adnexa (orbit, conjunctiva, and eyelids)a prospective multiparametric analysis of 108 cases during 1977 to 1987

    Hum Pathol

    (1990)
  • M.B. Moller et al.

    Testicular lymphoma: a population-based study of incidence, clinicopathological correlations and prognosis. The Danish Lymphoma Study Group, LYFO

    Eur J Cancer

    (1994)
  • D.C. Doll et al.

    Malignant lymphoma of the testis

    Am J Med

    (1986)
  • N. Nonomura et al.

    Malignant lymphoma of the testishistological and immunohistological study of 28 cases

    J Urol

    (1989)
  • H.D. Foss et al.

    Anaplastic large-cell lymphomas of T-cell and null-cell phenotype express cytotoxic molecules

    Blood

    (1996)
  • Z.I. Segal et al.

    Solitary extranodal anaplastic large cell lymphoma, Ki-1+, of the eyelid

    Am J Ophthalmol

    (1997)
  • R. Willemze et al.

    EORTC classification for primary cutaneous lymphomasa proposal from the Cutaneous Lymphoma Study Group of the European Organization for Research and Treatment of Cancer

    Blood

    (1997)
  • J.K. Chan et al.

    Nonnasal lymphoma expressing the natural killer cell marker CD56a clinicopathologic study of 49 cases of an uncommon aggressive neoplasm

    Blood

    (1997)
  • J.K. Chan et al.

    T- and T/natural killer-cell lymphomas of the salivary glanda clinicopathologic, immunohistochemical and molecular study of six cases

    Hum Pathol

    (1997)
  • I. Cochereau et al.

    Ocular involvement in Epstein-Barr virus-associated T-cell lymphoma

    Am J Ophthalmol

    (1996)
  • K. Peterson et al.

    The clinical spectrum of ocular lymphoma

    Cancer

    (1993)
  • L. Laroche et al.

    Immunological characterization of an ocular adnexal lymphoid T tumor by monoclonal antibodies

    Ophthalmologica

    (1983)
  • H. Brewitt et al.

    Lid und Orbitabeteiligung bei Mycosis fungoides

    Klin Monatsbl Augenheilkd

    (1974)
  • L. Durand et al.

    Localisation palpébrale d’un mycosis fongöide. Attitude thérapeutique

    Bull Soc Ophtalmol Fr

    (1971)
  • M.D. Sherman et al.

    Bilateral orbital infiltration as the initial sign of a peripheral T-cell lymphoma presenting in a leukemic phase

    Ann Ophthalmol

    (1990)
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