Pathophysiology: Primary site: Together with mucus-secreting goblet cells within the stratified epithelium, melanocytic cells exist in the basal layer of the conjunctiva. These melanocytic cells are of neuroectodermal origin, and melanocytic tumors may arise from these cells.

Melanomas may arise from junctional and compound nevi. Some uncertainty exists regarding the role of nevi in the histogenesis of malignant melanoma. Previously, compressed cells at the melanoma base have been considered to be nevi, but recent reports suggest that these flattened cells are, in fact, compressed melanoma cells and not nevus cells.

Melanomas also may arise from primary acquired melanosis or de novo.

Regional lymph nodes: The regional lymph nodes are parotid, preauricular, submandibular, and cervical.

For pN (see Staging), histologic examination of a regional lymphadenectomy specimen ordinarily includes 6 or more regional lymph nodes.

Metastatic sites: In addition to spread by lymphatics and the bloodstream, direct extension to the eyeball and orbit occurs.

Frequency:

• In the US: Primary malignant melanoma of the conjunctiva is much less common than intraocular or skin melanomas. Malignant melanoma of the conjunctiva accounts for only 2% of all ocular malignancies.

• Internationally: Denmark: Incidence of primary acquired melanosis (PAM) with atypia or with malignant melanoma of the conjunctiva was estimated to be 0.052 cases a year per 100,000.

  Sweden: Only 2 new cases of primary malignant melanoma of the conjunctiva were diagnosed in 1987.

Mortality/Morbidity: The overall tumor-related mortality rate for conjunctival melanoma is 25-26%. This rate increases to 40-44% if the tumor arose from PAM with an intraepithelial pagetoid growth pattern. This tumor tends to spread first to the parotid or submandibular nodes.

• Tumor thickness, mixed cell type, and lymphatic invasion on histologic examination were found to increase the death rate (3-4 times).

• Other poor prognostic features, outlined by Jakobiec and associates, include tumor in unfavorable locations (caruncle, palpebral conjunctiva, or fornical conjunctiva), moderate-to-severe atypia, a paucity of small polyhedral cells in the tumor, invasion of deeper ocular tissues, greater than 5 mitotic figures per 10 high-power fields, and lack of an inflammatory response induced by the tumor.

Race: This condition occurs predominantly in whites and rarely is seen in blacks.

Sex: No clear sex predilection has been established.

Age: Typically, conjunctival melanoma occurs in patients in their early 50s. It rarely is seen in people younger than 20 years. In 1999, Strempel reported 3 cases of conjunctival malignant melanoma in children.

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EYE REPUBLIC Ophthalmology Clinic Manila 3/F Don Santiago Building Units 309-310 1344 Taft Avenue, Ermita Manila, 1000 Philippines Direct and Fax: (632) 536-2398 Trunk Line: (632) 523-8271 to 79 local 30 Mobile: (63917) 899-2020 Map and directions	EYE REPUBLIC Ophthalmology Clinic Asian Hospital and Medical Center 5/F Medical Office Building (MOB) Suite 509 2205 Civic Drive, Filinvest, Alabang Muntinlupa City, 1781 Philippines Direct: (632) 771-9253 Direct and Fax: (632) 771-9254 Mobile: (63917) 795-2020 Map and Directions
EYE REPUBLIC Ophthalmology Clinic Medical City 6/F Medical Arts Tower Inc (MATI) Suite 602 MERALCO Compound, Ortigas Avenue Pasig City, 1604, Philippines Direct and Fax: (632) 632-7846 Mobile: (63917) 537-2020 Map and directions	EYE REPUBLIC Ophthalmology Clinic St. Luke's Medical Center 6/F Cathedral Heights Building Complex (CHBC) North Tower Suite 614 279 E. Rodriguez Sr. Boulevard Quezon City, 1102 Philippines Direct and Fax: (632) 407-3883 Mobile: (63917) 855-2020 Map and directions
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