Systemic B-celllymphoma with ocular manifestation and extraocular extension

SUMMARY The authors describe a case o f systemic lymphoma with ocular involvement as a histopathologic finding after the enucleation of a blind painful eye. The patient had chronic renal insufficiency and hypertension and had underwent multiple ophthalmologic surgical procedures. This paper discusses the histopatological aspects of the tumor, moreover the more frequent types of ocular lymphomas.


INTRODUCTION
The ocular presentation o f lympho mas is rare, and can be divided basi cally in its primary fo rm, where it attacks first the retina and vitreous and shows signs and symptoms o f an ocular uni or bilateral inflammation, and the secondary or systemic form, where it manifest itself as an uveal tumor, fr equently with an extraocular exten sion. It can be the systemic lympho ma's first manifestation. We can also differentiated this lymphomas accor ding to its cellular origin in T or B cells, with great precision by the im munohistochemistry.

Clinicai History
A 54-year-old caucasian man had been blind of his left eye since 1980. His past medicai history included hypertension and severa! ophthalmic procedures on his left eye: cataract surgery in 1971, and treatment o f a retina! detachment in 1978. In 1980, this eye was diagnosed as blind and atrophic. In July 1989, because a long standing renal insufficiency, a success fu l kidney transplant was performed. In 1992, the patient complained of pain in his blind eye, and an enuclea tion was performed.

Gross Pathology
The enucleated left eye measured, without including the extraocular mass, 25 x 24 x 24 mm with 3 mm attached optic nerve. The globe did not transilluminate. The cloudy cornea measured 1 O x 9 mm . When the eye was opened in the horizontal plane, the anterior chamber was fo und to be disorganized. The lens was absent. The uveal tract was thickened diffusely and infiltrated by a whitish, firm and homogeneous mass that also involved the episclera, particularly in the poste rior part of the eye (Fig. 1). An episcleral nodular mass, measuring 4 x 3 x 3 mm, was located approximately 3 mm temporally fr om the optic disc nerve head. There was no rupture of the sclera. The tissue comprising the episcleral nodule was identical to that infiltrating the uveal tract (Fig. 2).  Histologic sections of the left eye revealed a tumor composed of small, well-diffe rentiated lymphocytes diffu sely infiltrating the uveal tract and episclera in a nodular pattern (Fig. 3). The lymphocytes showed mild atypia; nucleoli were visible. Immunohisto chemical stains indicated that the vast ARQ. BRAS. OFTAL. 60(6), DEZEMBR0/1997 majority ofthe neoplastic cells (80% to 90%) were B-type cells displaying a monoclonal pattern of proliferation (Kappa positive), a finding that sup ported the diagnosis o f B-cell lympho ma (Fig. 4). Intraocular calcification, cholesterol granulomas, and a total retina! detachment were also observed. Immunostains fo r T cells (UCHL-1) showed that only 10% were T lympho cytes, ali of them being reactive, non neoplastic in nature.

Diagnosis
The diagnosis was well diffe ren tiated lymphocytic lymphoma of uveal tract, with extraocular extension. ted that ocular lymphomas occur in both a primary and secondary fo rm.

Primary Ocular Lymphoma
Primary ocular lymphoma involves the retina and vitreous. The patient may present with clinicai signs and symptoms of ocular inflammation; fr equently the initial diagnosis is bila teral uveitis o f unknown etiology. The disease is often associated with central nervous system (CNS) lymphoma. The immunohistochemical finding in both the ocular and CNS tumors is that of large B-cell lymphoma.
This group of tumors is widely referred to as reticulum cell sarcomas, a holdover fr om an earlier lymphoma nomenclature. They represent approxi mately 1% of non-Hodgkin's lympho mas fo und at all anatomical sites. Ty pically occurring in an elderly popu lation (median 60 years), primary in traocular lymphoma first appears as blurred vision and bilateral asymme tric posterior uveitis in patients who have no past history of systemic lym phoma 1 •2• Histopathologically, primary in traocular lymphoma usually infiltrate the retina in a multicentric fa shion and invade the vitreous with neoplastic and reactive cells. There is a striking ten dency for the neoplastic cells to invade the subretinal space, fo rming masses against the Bruch's membrane that  Os autores descrevem um caso de linfoma sistêmico, com manifestação ocular, como um achado histopatológico após a enuc/eação de um olho cego doloroso. O paciente apresentava insuficiência renal crônica e hipertensão, submetido a múltiplas cirurgias oft almológicas anteriores.