Ciralsky J, Colby K. Conjunctival melanomas: can the cancer stem cell hypothesis be applied? Semin Ophthalmol. 2009;24(3):161–165.
Esmaeli B. Regional lymph node assessment for conjunctival melanoma: sentinel lymph node biopsy and positron emission tomography. Br J Ophthalmol. 2008;92(4):443–445.
Kohanim S, Colby K. Evaluation and management of conjunctival melanoma. [American Academy of Ophthalmology Annual Meeting Video Program]. San Francisco: American Academy of Ophthalmology; 2011. Available at www.aao.org. Accessed December 20, 2012.
Shields CL, Shields JA, Gündüz K, et al. Conjunctival melanoma: risk factors for recurrence, exenteration, metastasis, and death in 150 consecutive patients. Arch Ophthalmol. 2000;118(11):1497–1507.
Neurogenic and Smooth-Muscle Tumors
Subconjunctival peripheral nerve sheath tumors such as neurofibromas, schwannomas, and neuromas have been reported, especially in multiple endocrine neoplasia (MEN). A neurofibroma of the conjunctiva or eyelid is almost always a manifestation of neurofibromatosis, an autosomal dominant phakomatosis (see BCSC Section 6, Pediatric Ophthalmology and Strabismus). A neurilemoma is a very rare tumor of the conjunctiva that originates from Schwann cells of a peripheral nerve sheath. A leiomyosarcoma is a very rare limbal lesion with the potential for orbital invasion.
Vascular and Mesenchymal Tumors
Vascular lesions of the eyelid margin or conjunctiva generally are benign hamartomas or secondary reactions to infection or other stimuli (Table 8-4).
Table 8-4
Benign Tumors
Hemangioma
A capillary hemangioma is usually present at birth and may enlarge slowly. Isolated capillary and cavernous hemangiomas of the bulbar conjunctiva are rare and are more likely to represent extension from adjacent structures. The palpebral conjunctiva is frequently involved with an eyelid capillary hemangioma. The presence of diffuse hemangiomatosis of the palpebral conjunctiva or conjunctival fornix indicates an orbital capillary hemangioma. A cavernous hemangioma of the orbit may present initially under the conjunctiva.
Nevus flammeus, a congenital lesion described as a port-wine stain, may occur alone or as part of Sturge-Weber syndrome, associated with vascular hamartomas, secondary glaucoma, and/or leptomeningeal angiomatosis. Some cases result from a genetic mutation coding for the vascular endothelial protein receptor for angiopoietin 1, which controls the assembly of perivascular smooth muscle. Ataxia-telangiectasia is a syndrome of epibulbar telangiectasis cerebellar abnormalities and immune alterations (see the section Lymphangiectasia and Lymphangioma).
Inflammatory vascular tumors
Inflammatory conjunctival lesions often show vascular proliferation. Pyogenic granuloma, a common
type of reactive hemangioma, is misnamed because it is not suppurative and does not contain giant cells. The lesion may occur over a chalazion or when minor trauma or surgery stimulates exuberant healing tissue with fibroblasts (granulation tissue) and proliferating capillaries that grow in a radiating pattern. This rapidly growing lesion is red, pedunculated, and smooth (Fig 8-12); it bleeds easily and stains with fluorescein dye. Topical or intralesional corticosteroids may be curative. Excision with cauterization to the base, primary closure of the wound, and generous postoperative topical corticosteroids may minimize recurrences.
Figure 8-12 Pyogenic granuloma (in association with a chronically inflamed chalazion). (Reproduced with permission from
Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. 3rd ed. Vol 1. Philadelphia: Elsevier/ Mosby; 2011:376.)
Subconjunctival granulomas may form around parasitic and mycotic infectious foci. They have also occurred with connective tissue diseases such as rheumatoid arthritis. Sarcoid nodules appear as tan-yellow elevations that can resemble follicles. Juvenile xanthogranuloma is a histiocytic disorder that can present as a conjunctival mass. A fibrous histiocytoma, composed of fibroblasts and histiocytes with lipid vacuoles, arises on rare occasions on the conjunctiva or limbus. Nodular
fasciitis is a very rare benign tumor of fibrovascular tissue in the eyelid or under the conjunctiva; it may originate at the insertion site of a rectus muscle. Necrobiotic xanthogranuloma is a very rare tumor that may affect the anterior orbit and eyelids. These lesions can present as subconjunctival or subdermal nodular fibrovascular tissue. Biopsy is essential to establish the diagnosis because this tumor is often associated with paraproteinemias, multiple myeloma, or lymphoma.
Malignant Tumors
Kaposi sarcoma
Kaposi sarcoma, a malignant neoplasm of vascular endothelium, involves the skin and mucous membranes. Internal organs are occasionally involved as well.
PATHOGENESIS Infection with Kaposi sarcoma–associated herpesvirus/human herpesvirus 8 (KSHV) is responsible for this disease. In young patients, it occurs most often in the setting of AIDS.
CLINICAL FINDINGS 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. Lesions are most often found in the inferior fornix and may be nodular or diffuse (Fig 8-13). Nodular lesions may be relatively less responsive to therapy.
Figure 8-13 Kaposi sarcoma of the conjunctiva. (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,