Kaposi sarcoma, a malignant neoplasm of vascular endothelium, involves the skin and mucous membranes. Internal organs are occasionally involved as well.
Infection with Kaposi sarcoma–associated herpesvirus/human herpesvirus 8 is responsible for this disease. In young patients, Kaposi sarcoma occurs most often in the setting of AIDS. Organ-transplant recipients and other highly immunosuppressed patients are at higher risk of developing Kaposi sarcoma.
On the eyelid skin, Kaposi sarcoma presents as a purplish nodule. Orbital involvement may produce eyelid and conjunctival edema. In the conjunctiva, Kaposi sarcoma presents as a reddish, highly vascular subconjunctival lesion that may simulate a subconjunctival hemorrhage. The lesions are most often found in the inferior fornix and may be nodular or diffuse (Fig 12-14). Nodular lesions may be relatively less responsive than diffuse lesions to therapy.
(Reproduced with permission from Holland GN, Pepose JS, Pettit TH, Gottlieb MS, Yee RD, Foos RY. Acquired immune deficiency syndrome. Ocular manifestations. Ophthalmology. 1983;90(8):859–873. Photograph courtesy of Gary N. Holland, MD.)
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.
Malignant mesenchymal lesions that infrequently involve the conjunctiva include malignant fibrous histiocytoma, liposarcoma, leiomyosarcoma, and rhabdomyosarcoma.
Excerpted from BCSC 2020-2021 series: Section 10 - Glaucoma. For more information and to purchase the entire series, please visit https://www.aao.org/bcsc.
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