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Clinicopathologic Reports, Case Reports, and Small Case Series
April 2002

Rapidly Progressive T-Cell Lymphoma of the Conjunctiva

Arch Ophthalmol. 2002;120(4):508-509. doi:

Conjunctival lymphoma classically presents as a salmon-colored infiltrate that evolves slowly over months to years.1 It is nearly always of B-cell lineage.1,2 In an analysis of ocular adnexal lymphoid tumors, 29% were polyclonal, 68% were monoclonal B-cell proliferations, 2% were indeterminate, 1% were null cell, and 0% were monoclonal T-cell proliferations.2 We report an unusual case of a rapidly progressive conjunctival mass that was the first manifestation of systemic T-cell lymphoma.

Report of a Case

A 72-year-old African American woman developed swelling of her left caruncle that dramatically enlarged throughout 21 days, prompting ocular examination. Her visual acuity was 20/200 OD and counting fingers OS from cataracts bilaterally. There was a painless, pink, multinodular mass occupying 60% of the bulbar surface from the caruncle to the lateral conjunctiva, and covering half of the cornea (Figure 1). Computed tomography scans revealed preseptal soft tissue swelling without an orbital component. The patient preferred 14 days' observation while receiving topical steroid/antibiotic medication, but the mass enlarged to affect 75% of the conjunctival surface, inducing lower eyelid ectropion. Diagnostic punch biopsy was performed.

Clinical course of conjunctival T-cell lymphoma. A, Throughout 21
days, there occurred a dramatic growth of the conjunctival stromal mass, involving
60% of the bulbar conjunctiva. B, Histopathological examination revealed poorly
cohesive pleomorphic cells, with prominent nuclei and scanty cytoplasm. Many
cells displayed folded or cleaved nuclei, suggestive of T cells (hematoxylin-eosin,
original magnification × 250). C, Following 3500 rad (35 Gy) of radiotherapy,
the mass completely regressed.

Clinical course of conjunctival T-cell lymphoma. A, Throughout 21 days, there occurred a dramatic growth of the conjunctival stromal mass, involving 60% of the bulbar conjunctiva. B, Histopathological examination revealed poorly cohesive pleomorphic cells, with prominent nuclei and scanty cytoplasm. Many cells displayed folded or cleaved nuclei, suggestive of T cells (hematoxylin-eosin, original magnification × 250). C, Following 3500 rad (35 Gy) of radiotherapy, the mass completely regressed.

Histopathological examination revealed a diffuse infiltrate of poorly cohesive medium to large cells, with moderate prominent nucleoli, and moderate, sometimes eccentric, cytoplasm. Many cells displayed folded or cleaved nuclei. Germinal centers were absent, and numerous mitotic figures were present. Immunohistochemical stains for leukocyte common antigen (CD45), UHCL-1 (CD45Ro), and CD8 were positive, suggestive of a lymphoid neoplasm of T-cell origin. B-cell marker, L26 (CD20), was negative. The final diagnosis was peripheral T-cell lymphoma.

A thorough sytemic examination revealed no systemic lymphoma or mycosis fungoides. The patient was treated with 3500 rad (35 Gy) of radiotherapy to the left conjunctiva with rapid regression of the lymphoma (Figure 1). During her last week of radiotherapy, she experienced dysphagia and a 2.72 kg weight loss. She was discovered to have an obstructive nasopharyngeal mass, and cervical, inguinal, and popliteal lymphadenopathy. On examination of the biopsy specimen, the nasopharyngeal mass proved to be T-cell lymphoma, and radiotherapy (1300 rad [13 Gy]) was delivered to the site, and there was improvement. Systemic chemotherapy, using cytoxan, vincristine, and adriamycin, was administered for 2 cycles. The patient maintained poor follow-up for 11 months, and she subsequently died of an unknown cause.

Comment

Lymphomas can be broadly classified into Hodgkin and non-Hodgkin types. Non-Hodgkin lymphomas are a diverse group and include monoclonal neoplasms of B-cell or T-cell lymphocytes. T-cell lymphomas are much less common than B-cell lymphomas and are particularly rare in the ocular region.1,2 Of the few reported cases of ophthalmic T-cell lymphomas, most involved the orbit or eyelid, and followed known systemic T-cell lymphoma.3,4 Rapid progression throughout days to weeks, and poor systemic prognosis was a common feature. Systemically, however, T-cell lymphoma can progress rapidly in aggressive subtypes or it can progress slowly as in mycosis fungoides. Cook and associates reviewed 2155 patients at the Mayo Clinic with known cutaneous T-cell lymphoma, and found infiltration of the eyelid in 0.3%, and conjunctiva in 0.05%.4 Thus, ocular involvement with T-cell lymphoma is exquisitely rare.

Using a MEDLINE search, we found one other case of T-cell lymphoma that initially presented in the conjunctiva, similar to our case.5 The conjunctival tumor in that case also showed rapid progression. The patient was a 63-year-old woman who presented with bilateral limbal conjunctival thickening and chemosis for 20 days, and histopathologic study revealed T-cell lymphoma.5 Our patient also had a rapidly enlarging conjunctival mass that began as a caruncular thickening and extended in 21 days to involve 60% of the bulbar conjunctiva. Both patients were found subsequently to have systemic T-cell lymphoma, with involvement of maxillary sinus in their case, and nasopharynx in our case. Thus, systemic evaluation is particularly important in these cases. In summary, we present an unusual case of a rapidly progressive salmon-colored conjunctival tumor that proved to be the first sign of sytemic T-cell lymphoma.

Support was provided by the Eye Tumor Research Foundation, Philadelphia, Pa (Dr C. L. Shields); the Paul Kayser International Award of Merit in Retina Research, Houston, Tex (Dr J. A. Shields); and the Noel T. and Sara L. Simmonds Endowment for Ophthalmic Pathology, Wills Eye Hospital, Philadelphia (Dr Eagle).

Corresponding author: Carol L. Shields, MD, Ocular Oncology Service, Wills Eye Hospital, 900 Walnut St, Philadelphia, PA 19107.

References
1.
Shields  CLShields  JACarvalho  CRundle  PSmith  AF Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 2001;108979- 984Article
2.
Jakobiec  FAKnowles  DM An overview of ocular adnexal lymphoid tumors. Trans Am Ophthalmol Soc. 1989;87420- 442
3.
Coupland  SEFoss  HDAssaf  C  et al.  T-cell and T/natural killer-cell lymphomas involving ocular and ocular adnexal tissues: a clinicopathologic, immunohistochemical, and molecular study of seven cases. Ophthalmology. 1999;1062109- 2120Article
4.
Cook  BEBartley  GBPittelkow  MR Ophthalmic abnormalities in patients with cutaneous T-cell lymphoma. Ophthalmology. 1999;1061339- 1344Article
5.
Hu  FRLin  JCChiang  IPYeh  KH T-cell malignant lymphoma with conjunctival involvement. Am J Ophthalmol. 1998;125717- 719Article
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