The management of conjunctival melanoma is difficult because of the rarity of the disease. It is an uncommon but potentially devastating tumor that may invade the local tissues of the eye, spread. Conjunctival melanoma is an ocular surface cancer, and as so, it differs substantially in its features and management from the previously. Surgical resection of conjunctival squamous cell carcinoma using the no touch. This was because pam with atypia doesnt define it as a cancer. Systemic treatment of metastatic conjunctival melanoma. 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.
An incidence of 2% is reported in the united states. Conjunctival melanoma arises denovo as a result ofmalignant change occuring in melanocytes. Conjunctival melanoma in childhood and adolescence. Pigmented conjunctival lesions exhibit a wide variety of appearances, and the differential diagnoses of these include nevi, melanoses, and malignant melanomas.
It is potentially lethal, with regional lymph node spread often preceding distant solidorgan. Research open access oncologic and visual outcomes after postoperative proton therapy of localized conjunctival melanomas juliette thariat1,2, julia salleron3, celia maschi4, edouard. Conjunctival melanoma is a disease of middleaged and elderly persons, with very few cases reported in children. Conjunctival melanoma is an ocular surface cancer, and as so, it differs substantially in its features and management from the previously described intraocular tumours. A novel kras mutation in metastasic conjunctival melanoma. It is a rare malignancy, accounting for 12% of all ocular melanomas. Conjunctival melanoma is a rare malignant condition of the ocular surface. Conjunctival melanomatreatmentorbital exenteration. The efficacy of tld1433 was tested on several cell lines from cm crmm1, crmm2 and cm2005, uveal. It accounts for 2%5% of ocular malignancies1 and 5%7% of all primary ocular melanoma. What is the cause and treatment for conjunctival malignant. A rare case of malignant conjunctival melanoma is presented.
Full text surgical management of a conjunctival nevus. The problems encountered in diagnosis and management are discussed. Melanocytic lesions of the conjunctiva archives of. However, in recent years a significant increase in the number of cases of conjunctival melanoma has been reported in males with the incidence of conjunctival melanoma remaining unchanged. Objectiveto identify the risk factors of conjunctival malignant melanoma that. Reprinted from university of utah, peter debry, kwok li, and nick mamalis, md, ophthalmic pathology. Conjunctiva tumor an overview sciencedirect topics. However, it also generates free radicals that damage dna and proteins through. The rutheniumbased photosensitizer ps tld1433 has completed a phase i clinical trial for photodynamic therapy pdt treatment of bladder cancer. Conjunctival melanoma is an uncommon malignancy with the potential for significant morbidity. Abstract conjunctival malignant melanoma is a rare disease associated with. Here, we investigated a possible repurposing of this drug for treatment of conjunctival melanoma cm.
Melanocytic proliferations are the most common tumors of the conjunctiva, accounting for up to 53% of all conjunctival neoplasms. In their recent study, griewank and colleagues proposed that new therapies effective in cutaneous melanoma could hold promise in the management of conjunctival. 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. Research open access oncologic and visual outcomes after postoperative proton therapy of localized conjunctival melanomas juliette thariat1,2, julia salleron3, celia maschi4, edouard fevrier4, sandra lassalle5, lauris gastaud6.
It is now commonly used as adjuvant therapy after excisional biopsy and. Sometimes it starts as a painless, flat brown discoloration of. Conjunctival epithe lium with underlying tissue infiltrated by tu mour cells admixed with inflammatory cells. The surgical technique of complete excision with a wide tumorfree margin of 4.
In 6067 90% of the conjunctival melanoma cases, both test types were negative. Tld1433 photosensitizer inhibits conjunctival melanoma cells. Here, we present the case of a 48yearold patient with conjunctival melanoma and metachronic lymph node and skin metastasis with kras p. Early diagnosis facilitates complete excision of the tumour with goodprognosis. Conjunctival melanoma presents similarities with cutaneous melanoma, with substantial differences in the biological behavior. Conjunctival melanoma pictures things you didnt know. Two late recurrences of conjunctival melanoma fulltext ocular. Doctor answers on symptoms, diagnosis, treatment, and more. The efficacy of tld1433 was tested on several cell lines from cm crmm1, crmm2 and cm2005, uveal melanoma omm1, omm2. Invasive melanoma of the conjunctiva is a rare but sight and lifethreatening condition. It is potentially lethal, with regional lymph node spread often preceding distant solidorgan metastasis. Conjunctival melanoma com constitutes about 2% of all ocular malignancies and 5% of all ocular melanoma. Oncologic and visual outcomes after postoperative proton.
Note in a multicenter international effort to develop a staging system for conjunctival melanoma, the authors. The management of metastatic uveal melanoma is discussed separately. Conjunctival melanomas harbor braf and nras mutations. Conjunctival melanoma arises when melanocytes in the conjunctival epithelium become malignant. Pdf management of conjunctival melanoma sarah coupland. Conjunctival melanomas harbor braf and nras mutations and. Melanoma of the conjunctive confined to the epithelium pt1a. A conjunctival nevus and benign acquired melanosis can be photographed and followed for evidence of change prior to intervention. Backgroundobjective in patients with conjunctival melanomas, surgery is the first choice of treatment, but no standard adjuvant therapy has been established. Once the tumor has metastasized, treatment options are limited and the prognosis is poor. An uncommon eye cancer that arises in the conjunctiva, the clear membrane that covers the outer surface of the eye and the inner part of the.
Conjunctival melanoma and melanocytic intraepithelial. The management of conjunctival melanoma requires expert care from an experienced, multidisciplinary team. Conjunctival malignant melanoma is a pigmented lesion of the ocular surface. Uveal melanomas can arise in any part of the uveal tract, including the iris, ciliary body, and choroid. These malignant melanocytes can multiply and spread within the epithelium. The most common conjunctival cancers are squamous carcinoma, malignant melanoma and lymphoma. They may involve any area of the conjunctiva including the bulbar conjunctiva, plica and caruncle areas covering the surface of the eye itself, where they are more easily noticeable or involve areas such as fornix and tarsal conjunctiva, which are hidden behind the eyelid and may not be. Sometimes it starts as a painless, flat brown discoloration of the surface of the eye, termed primary acquired melanosis. Conjunctival melanoma typically presents as a pigmented nodular or flat. Due to its rarity and the long latency between treatment and local recurrence or the development of metastases, it is difficult to study. Conjunctival melanosis and melanoma robert folberg, md ian w. However, in recent years a significant increase in the number of cases of conjunctival melanoma has been reported in males with the incidence of conjunctival melanoma remaining unchanged in females. Despite effective local therapies, recurrence and metastasis remain frequent.
Conjunctival primary acquired melanosis pam accounts for 11% of all conjunctival tumors and 21% of melanocytic lesions. This conjunctival melanoma likely developed from primary acquired melanosis pam with atypia and then grew onto the superficial surface of the cornea. Melanoma of the palpebral, forniceal or caruncular conjunctiva more than 0. The melanoma was removed with tumorfree margins fig.
Conjunctival melanoma is more common in individuals with a fair complexion with a tendency to freckling and sunburn. Conjunctival melanoma is a rare but potentially deadly ocular malignancy. Conjunctival melanoma british journal of ophthalmology. This is often mistaken for a benign freckle, but actually is a serious precancerous condition. Conjunctival melanoma cjm is a rare, primary cancer of the ocular region.
The lesion is removed with a tumor free margin of 4 to 5 mm. Conjunctival melanoma is a rare but potentially deadly tumor of the eye. The initial management of uveal and conjunctival melanoma is discussed in this section. To date, little is known of the genetic alterations in conjunctival melanomas. A rare conjunctival melanoma case by christopher weyer, do, elizabeth young, and leslie marshall, do summary primary conjunctival melanomas.
Management of primary acquired melanosis, nevus, and conjunctival melanoma andrew kao, md, armin afshar, md, michele bloomer, md, and bertil damato, md, phd photo courtesy of lynn e. Malignant melanoma used to be called pam with atypia, but the newest ajcc staging system authors suggest we stop using the term pam with atypia and call it melanoma in situ. These are better evaluated in person so that subtle changes can be seen. It accounts for 2%5% of ocular malignancies1 and 5%7% of all primary ocular. A clinicopathologic study of 41 cases of primary acquired melanosis has provided information concerning the rate of progression to melanoma and has provided histologic parameters useful in predicting which lesions are likely to progress to malignant melanoma. Doublefreeze, slowthaw cryotherapy is applied to the conjunctival edges. Conjunctival melanoma cm is a rare malignancy originating from atypical melanocytes in the conjunctival or limbal epithelium and comprises 2% of all ocular melanomas mccartney 1995. It is an uncommon but potentially devastating tumor that may invade the local tissues of the eye, spread systemically through lymphatic drainage and hematogenous spread, and recur in spite of treatment. Management of primary acquired melanosis, nevus, and. Page 3 tumors nevus a benign pigmented conjunctival tumor of the conjunctiva. For example, the terminology of conjunctival melanocytic lesions. The patient had negative sentinel lymph node biopsy and has been disease free. Excision with cryotherapy is the preferred treatment for pam with atypia.
If you have problems viewing pdf files, download the latest version of adobe reader. Diagnosis and management of conjunctival melanoma american. Conjunctival melanoma arising from primary acquired. It is associated with a mortality of 2530% norregaard et al. Tm these tumors are primarily related to ultraviolet uv exposure.
In the present study, we report a male to female ratio of 1. Conjunctival melanoma is a rare but potentially deadly ocular. Genetic and epigenetic characteristics of conjunctival melanoma have not been completely elucidated yet. Conjunctival melanocytic tumors are fairly common, representing approximately 53% of cases in one large series of 1643 tumors and 52% in a. Conjunctival melanoma genetic and rare diseases information. The goal of therapy is to provide good local tumor control. Malignant conjunctival melanoma and intraepithelial melanoma in situ should be removed or destroyed. Advances in the management of conjunctival melanoma ncbi. Conjunctival melanoma are usually pigmented and raised. Suspicious neoplastic conjunctival lesions often require wide excision with tumour free margins, leaving significant conjunctival defects requiring reconstruction. Surgical resection of conjunctival squamous cell carcinoma using the no touch technique duration.
Finger says, think of sunglasses as sun block for your eyes. Melanoma in situ and invasive conjunctival melanoma new. Conjunctival melanoma is a rare disease, and little is known about its molecular background. Ulceration is known to be an indicator of more aggressive cutaneous melanoma. They may involve any area of the conjunctiva including the bulbar conjunctiva, plica and caruncle areas covering the surface of. Gard po box 8126, gaithersburg, md 208988126 toll free. Here, we present the case of a 48yearold patient with conjunctival melanoma and.
The literature is composed entirely of case series of varying quality, and. Melanoma of the palpebral, forniceal or caruncular conjunctiva not more than 0. The treatment of conjunctival melanoma is surgical, with complete removal of the tumour, if possible the melanoma is usually removed using a notouch technique, followed by freezing. Tld1433 photosensitizer inhibits conjunctival melanoma. For the case reports, 17 cases were disease free at the final followup, with a median followup duration of 1. The ozone layer is thinning and the incidence of conjunctival melanoma is increasing. This is a pdf file of an unedited manuscript that has. In this study, we evaluated prognostic factors for conjunctival melanoma in ethnic chinese patients. Conjunctival melanoma is rare in adults and rarer in children. An internet ocular pathology archive, with permission from university of utah ophthalmic pathology. Conjunctival melanoma is a cancerous, lifethreatening growth that typically occurs as a painless brown spot on the eye in midlife. Methods demographic data, known published prognostic factors, braf and kit gene mutations, treatment strategies and outcomes were.