MD.)

MANAGEMENT Treatment may not be curative. Options for controlling symptoms include surgical debulking, cryotherapy, and radiotherapy. Local or systemic chemotherapy may be required. Intralesional interferon-α2a has been reported to be effective.

Other malignant tumors

Malignant mesenchymal lesions that infrequently involve the conjunctiva include malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, and rhabdomyosarcoma.

Lymphatic and Lymphocytic Tumors

Lymphoid tumors of the conjunctiva may be benign, malignant, or indeterminate. Many of these lesions have overlapping clinical and pathologic features. Approximately 20% of patients with a conjunctival lymphoid tumor have detectable extraocular lymphoma.

Lymphangiectasia and Lymphangioma

Lymphangiectasia appears in the eye as irregularly dilated lymphatic channels in the bulbar conjunctiva. It may be a developmental anomaly or can follow trauma or inflammation. Anomalous communication with a venule can lead to spontaneous filling of the lymphatic vessels with blood.

Lymphangiectasia must be distinguished from ataxia-telangiectasia (Louis-Bar syndrome), in which the epibulbar and interpalpebral telangiectasia of the arteries lacks an associated lymphatic component. The epibulbar vascular lesions of Louis-Bar syndrome can grow with the patient and the eyeball, but episodes of hemorrhage or swelling do not occur. Ataxia-telangiectasia is discussed and illustrated in greater detail in BCSC Section 6, Pediatric Ophthalmology and Strabismus.

Lymphangiomas are proliferations of lymphatic channel elements. Like a capillary hemangioma, a lymphangioma is usually present at birth and may enlarge slowly. The lesion appears as a patch of vesicles with edema. Intralesional hemorrhage, producing a “chocolate cyst,” makes differentiation from a hemangioma difficult.

Lymphoid Hyperplasia

PATHOGENESIS Formerly called reactive hyperplasia, this benign-appearing accumulation of lymphocytes and other leukocytes may represent a low-grade B-cell lymphoma. Most patients are older than 40 years, although, in rare instances, extranodal lymphoid hyperplasia has occurred in children.

CLINICAL FINDINGS Lymphoid hyperplasia presents as a minimally elevated, salmon-colored subepithelial tumor with a pebbly appearance corresponding to follicle formation (Fig 8-14); it is clinically indistinguishable from conjunctival lymphoma. It is often moderately or highly vascularized. Primary localized amyloidosis can have a similar appearance.

Figure 8-14 Conjunctival lymphoid hyperplasia.

MANAGEMENT Lymphoid hyperplasia may resolve spontaneously, but these lesions have been treated with local excision, topical corticosteroids, or radiation. Biopsy specimens require special handling to complete many of the histochemical and immunologic studies. Fresh tissue is required for immunohistochemistry, flow cytometry, and gene rearrangement studies. Because a patient with an apparently benign polyclonal lymphoid lesion has the potential to develop a systemic lymphoma, general medical consultation is advisable.

Lymphoma

A neoplastic lymphoid lesion of the conjunctiva is generally a monoclonal proliferation of B lymphocytes.

PATHOGENESIS A lymphoma can arise in conjunctival lymphoid follicles. Some lymphomas are limited to the conjunctiva; others occur in conjunction with systemic malignant lymphoma. Some are polyclonal, but most conjunctival lymphomas are monoclonal B-cell lymphomas. Conjunctival plasmacytoma, Hodgkin lymphoma, and T-cell lymphomas are less common.

CLINICAL FINDINGS Non-Hodgkin B-cell lymphoma has essentially the same clinical appearance as benign lymphoid hyperplasia. It appears as a salmon pink, mobile mass on the conjunctiva (Fig 8-15). The lesions are usually unilateral; however, 20% are bilateral. A diffuse lesion may masquerade as

chronic conjunctivitis. An epibulbar mass fixed to the underlying sclera may be a sign of extrascleral extension of uveal lymphoid neoplasia. Most patients with conjunctival lymphoma are either older than 50 years or immunosuppressed.

Figure 8-15 Conjunctival lymphoma.

LABORATORY EVALUATION AND MANAGEMENT Patients should be referred to an oncologist for systemic evaluation, as up to 31% of patients may have or may eventually develop underlying systemic lymphoma. Unless a tumor is small enough to be removed completely, incisional biopsy is indicated for histologic diagnosis. Local external-beam radiation therapy is usually curative for lesions confined to the conjunctiva, but systemic chemotherapy is required for the treatment of systemic lymphoma. Cryotherapy and intralesional chemotherapy with interferon-α2b have also been described.

Shields CL, Shields JA, Carvalho C, et al. Conjunctival lymphoid tumors: clinical analysis of 117 cases and relationship to systemic lymphoma. Ophthalmology. 2001;108(5):979–984.

Warner MA, Bhat PV, Jakobiec FA. Subepithelial neoplasms of the conjunctiva. In: Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. 3rd ed. Vol 1. Philadelphia: Elsevier/Mosby; 2011:485–507.