Management of conjunctival malignant melanoma: a review and update

Abstract

Conjunctival malignant melanoma is a pigmented lesion of the ocular surface. 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. Despite its severity, the rarity of available cases has limited the evidence for diagnosis and management. This review will provide an overview of the epidemiology, risk factors, presentation, diagnosis, management, prognosis, staging, and surveillance of conjunctival melanoma, with an emphasis on recent advances in biological therapies to treat this disease.

Keywords::

• BRAF inhibitors
• conjunctival malignant melanoma
• dabrafenib
• IFNα-2β
• ipilimumab
• melanoma
• mitomycin C
• ocular neoplasm
• vemurafenib

Acknowledgements

The authors would like to thank Sander R. Dubovy and J. Antonio Bermudez-Magner, eliminated just to be consistent for graciously providing representative histopathology images.

Financial & competing interests disclosure

The authors were supported by NIH Center Core Grant P30EY014801, Research to Prevent Blindness Unrestricted Grant, Department of Defense (DOD- Grant#W81XWH-09-1-0675), The Ronald and Alicia Lepke Grant, The Lee and Claire Hager Grant, The Jimmy and Gaye Bryan Grant. Galor is supported by the Department of Veterans Affairs, Veterans Health Administration, Office of Research and Development, Clinical Sciences Research and Development's Career Development Award CDA-2-024-10S. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Conjunctival melanoma is an uncommon but increasingly important and potentially fatal tumor of the eye.

• Prognosis is poor, with recurrence and metastasis commonly occurring despite excision with tumor-free margins. Recurrences are associated most strongly with a nonlimbal location. The percentage of patients with local metastasis, distant metastasis and melanoma-related death approach 52%, 42% and 23%, respectively, within 5 years.

• Diagnosis is generally by clinical examination and slit-lamp findings. However, high-resolution imaging such as high-resolution optical coherence tomography and confocal microscopy may offer assistance with making the diagnosis, and assisting in surgical planning.

• Current management is wide local excision with cryotherapy to the margins. Adjuvant therapies include topical chemotherapy, cryotherapy and radiotherapy.

• Follow-up should include referral to an oncologist. In spite of some controversy, lymphoscintigraphy should be performed for tumors >2 mm in thickness.

• Advances in characterizing conjunctival melanoma at a genetic level are offering insight into potential biological therapies that are already in use for the treatment of cutaneous melanoma. This is the new frontier for management of conjunctival melanoma.

Notes