Conjunctival melanoma is a rare but potentially deadly ocular. Management of ocular melanoma whole final document sept. Systemic treatment of metastatic conjunctival melanoma. The initial management of uveal and conjunctival melanoma is discussed in this section. Conjunctival melanoma cm is an exceptionally rare tumor, with a propensity for local and distant recurrence, with the lungs, skin, liver, and brain being the most common sites of metastasis. Malignant melanoma can start as a conjunctival nevus, arise as newly formed pigmentation or variably pigmented within the conjunctiva or onto the cornea. Conjunctival melanoma is more common in individuals with a fair complexion with a tendency to freckling and sunburn. It arises from melanocytes located amongst the basal cells of the conjunctival epithelium.
A new cell line from a recurrent conjunctival melanoma. Diagnosis and management of conjunctival melanoma american. Conjunctival melanoma ajcc cancer staging manual, 8th edition copyright 2016 american joint committee on cancer. An analysis of 410 patients with conjunctival nevus who were followed for a mean of seven years showed that 1% of the patients were eventually diagnosed with melanoma. Conjunctival epithe lium with underlying tissue infiltrated by tu mour cells admixed with inflammatory cells. Invasive melanoma of the conjunctiva is a rare but sight and lifethreatening condition. The conjunctiva is the clear tissue that covers the white part of the eye, as well as the inside of the eyelids.
Malignant conjunctival melanoma and intraepithelial melanoma in situ should be removed or destroyed. Coms clinical trials are funded by the national eye institute and the national cancer institute of the national institutes of health, and are being conducted at more. Links to pubmed are also available for selected references. Pdf systemic treatment of metastatic conjunctival melanoma. In the 18 cases in which exenteration was required, because of the unfavourable localization of the melanoma or repeated recurrences, the. Conjunctival melanoma melanoma research foundation. Dataset for the histopathological reporting of conjunctival melanoma.
Journal of pediatric ophthalmology and strabismus introductionthe annual incidence of conjunctival melanoma in the united states has been estimated to be 0. Conjunctival melanoma are usually pigmented and raised. Uveal melanomas can arise in any part of the uveal tract, including the iris, ciliary body, and choroid. Conjunctival melanoma often appears as a raised tumor and may contain little or no pigment. Conjunctival tumors are one of the most frequent of. Nodular multifocal conjunctival melanoma in a 30yearold black woman was treated using surgical excision and adjuvant cryotherapy. Malignant melanoma is a potentially fatal tumor that arises from melanocytes, most often in the sunexposed skin. Coms clinical trials are funded by the national eye institute and the national cancer institute of the national institutes of health, and are being conducted at more than 40 medical centers in the united states and canada. Of the two, uveal melanoma is more common, with an incidence of four to six per million per year in most western countries. In 1980, the who proposed the term pam to describe this disorder.
The two principal types of primary ocular melanoma are uveal and conjunctival melanoma. Conjunctival malignant melanoma cmm is a rare but potentially lifethreatening cancerous growth of the eye. Recent progress in systemic treatments, with checkpoint inhibitors and targeted therapies blocking braf and mek, has redefined the standard of care of advanced unresectable and metastatic melanoma. These proposals for the reporting of conjunctival melanoma and melanosis should be. It accounts for 2%5% of ocular malignancies1 and 5%7% of all primary ocular melanoma. Advances in the management of conjunctival melanoma survey of. It can arise in previously unblemished and unpigmented regions approximately 10% of cases, from a preexisting nevus approximately 20% of cases, or from the flat, spreading pigmentation of primary acquired melanosis with atypia 6070% of cases. The management of metastatic uveal melanoma is discussed separately. Clinical predictive factors for development of recurrence and metastasis in conjunctival melanoma. Pdf conjunctival melanoma cm is an exceptionally rare tumor, with a propensity for local and distant recurrence, with the lungs, skin, liver, and. One might expect as many or more problems in studying conjunctival melanoma because it is a rare disease, occurring approximately one fortieth as often as choroidal melanoma. Melanocytic lesions of the conjunctiva archives of. Note in a multicenter international effort to develop a staging system for conjunctival melanoma, the authors agreed that the term primary acquired melanosis with atypia should be abandoned in favor of conjunctival melanoma in situ. Management of ocular melanoma whole final document sept 2008.
Normally, it contains melanocytes which explains possibility of development of pigmented tumors in the conjunctiva. Sep 08, 2017 malignant melanoma of the conjunctiva presents as a raised pigmented or nonpigmented lesion. Invasive conjunctival melanomas have an inci dence of less than one per million per year 1. Braf mutations have been detected in up to 50% of primary and metastatic conjunctival melanomas as in cutaneous melanoma,2834,51. A conjunctival malignant melanoma shows invasion of melanoma cells into the subepithelial layers. Tumores conjuntivales, neoplasia intraepitelial, nevus, melanoma, linfoma. Treatment of conjunctival malignant melanoma with topical. Melanoma in situ and invasive conjunctival melanoma new. Sometimes it starts as a painless, flat brown discoloration of the surface of the eye, termed primary acquired melanosis. A juvenile case of conjunctival atypical nevus diagnostic.
Conjunctival melanoma is a rare but potentially fatal ocular surface tumor affecting about 0. This conjunctival melanoma likely developed from primary acquired melanosis pam with atypia and then grew onto the superficial surface of the cornea. Conjunctival melanoma arising from primary acquired melanosis. Conjunctival melanoma is a cancerous, lifethreatening growth that typically occurs as a painless brown spot on the eye in midlife. Conjunctival melanoma arises denovo as a result ofmalignant change occuring in melanocytes. So far only three conjunctival melanoma cell lines have been described. Uveal melanomas are rare, affecting about six persons per million per year. A simple biopsy can determine whether a pigmented conjunctival tumor is a benign nevus, primary acquired melanosis, or conjunctival melanoma. Objectiveto identify the risk factors of conjunctival malignant melanoma that predict local tumor recurrence, orbital exenteration, distant metastasis, and tumo. Recognition of their precursor lesions at an early stage is. Apr 22, 20 among conjunctival tumors, melanocytic lesions represent 53% of all excised conjunctival lesions. Malignant melanoma of the conjunctiva can arise from a conjunctival nevus. Full text full text is available as a scanned copy of the original print version.
Intraocular melanomas arise in the uvea, which is a deeply pigmented tissue within the eye. The authors describe the clinicopathological features of four mexican patients with advanced conjunctival melanoma. Sixty percent of conjunctival melanomas arise from primary acquired melanosis pam and 20% from nevi. Conjunctival melanoma metastasis diagnosed by sentinel lymph node. Pdf ocular melanoma is the second most common type of melanoma after cutaneous and the most common primary intraocular malignant tumor in adults. Manual search through references from these articles was also done. An incidence of 2% is reported in the united states. Conjunctival melanoma is an uncommon tumor that is likely to recur and carries an overall mortality rate of approximately 30%. Nevi are the most common, found in 52% of the cases, followed by melanoma in 25%, primary acquired melanosis pam in 21%, and racial melanosis in 3%. Melanocytic proliferations are the most common tumors of the conjunctiva, accounting for up to 53% of all conjunctival neoplasms. It is an uncommon tumor which comprises about 2% of all eye tumors, 5% of melanomas in the ocular region and 0. Conjunctival melanoma and pri mary acquired melanosis pam are pigmented lesions of the ocular surface. Conjunctival malignant melanoma is a pigmented lesion of the ocular surface.
This is often mistaken for a benign freckle, but actually is a serious precancerous condition. Conjunctival melanoma is a rare neoplasm with an aggressive behaviour characterized by a high tendency to develop recurrences and metastases. They tend to present in adulthood and the median age at. Biopsy can determine whether a pigmented conjunctival tumor is a nevus, primary acquired melanosis, or conjunctival melanoma. Extensive pam with severe atypia, including areas of microinvasive melanoma, was treated using.
The collaborative ocular melanoma study or coms for short, is a set of clinical trials designed to evaluate the treatments doctors use for their patients with choroidal melanoma. Pam can involve a small spot on the conjunctiva or extend to cover the. Malignant melanoma of the conjunctiva sage journals. Conjunctival melanoma treated with complete circumferential peripheral and deep margin assessment slow mohs surgery.
With a 30% mortality rate and an ability to easily metastasize, conjunctival melanoma is one of the most feared lesions a person can have, dr. The seemingly unpredictable and enigmatic character of this entity has initiated much debate over the past decades regarding the etiology, histogenesis, prognosis, and preferred management. In a followup study of 45 conjunctival melanoma patients the recurrence rate after local excision was 80% but the mortality rate due to metastasis was low, only 2 out of 25 cases in which the recurrences could be controlled by repeated local excisions. There are some lesions that cannot be unequivocally assigned to a nevus, pam, melanoma. Conjunctival melanoma most commonly occurs in the bulbar conjunctiva the mucous membrane that covers the outer surface of the eyeball.
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