Melanoma on the Cornea: Risk of Metastatic Disease
Melanoma on the cornea is not contained and the individual remains at risk for metastatic disease, requiring appropriate surveillance and management.
Understanding Ocular Melanoma
- Ocular melanoma is the second most common type of melanoma after cutaneous melanoma and the most common primary intraocular malignant tumor in adults 1
- Conjunctival melanoma (which includes corneal involvement) is rare but has an increasing incidence, occurring mostly among white adults 1
- In the majority of cases, conjunctival melanoma originates from preceding primary acquired melanosis 1
Risk of Metastatic Disease
- Conjunctival melanoma tends to spread to other adnexal structures and has metastatic potential 2
- Patients with ocular melanomas, including those affecting the cornea, remain at risk for metastatic disease even after successful local treatment 3
- Approximately 50% of patients with ocular melanoma develop metastatic disease despite successful eradication of the primary tumor 3
- The prognosis of patients with metastatic ocular melanoma remains poor due to lack of effective systemic therapy 1
Diagnostic Considerations
- Melanoma on the cornea typically presents as a painless, flat or nodular, brown or fleshy-pink lesion 2
- Enlargement of the lesion, presence of blood vessels, or thickening often trigger an office visit 2
- Diagnosis is typically established by clinical examination with great accuracy in most cases 1
Risk Factors
- Significant UV exposure is a risk factor for conjunctival melanoma 2
- Previous history of melanoma increases risk 2
- Previous primary acquired melanosis or Nevus of Ota are predisposing factors 2
- Fair skin that sunburns easily and inability to tan are associated with increased risk 2
Treatment Approaches
- Current standard treatment for conjunctival melanoma is wide local excision with adjuvant therapy 1
- Adjuvant therapies include brachytherapy, cryotherapy, and topical application of chemotherapeutic agents 1
- Local treatment of ocular melanoma has improved with increased use of conservative methods and preservation of the eye 1
- Despite improved local control, survival rates have remained largely unchanged 1
Surveillance and Follow-up
- Lifetime dermatologic surveillance is recommended for all patients with melanoma due to the risk of metastatic disease 2
- The frequency of dermatologic surveillance should be determined individually based on risk factors 2
- Skin examination and surveillance at least once a year for life is recommended 2
- For patients with metastatic disease, treatment of the CNS disease usually takes priority to prevent complications 2
Prognostic Factors
- Recent advances in cytogenetics and genetics have enhanced prognostication and enabled determination of tumors with high metastatic potential 1
- Metastatic disease remains the leading cause of death among patients with ocular melanoma 1
- Metastases from ocular melanoma are most frequently localized to the liver 4
Clinical Implications
- Better understanding of underlying genetic and molecular abnormalities in ocular melanomas provides opportunities for development of targeted therapy 1
- As with other melanomas, early detection and treatment could be critical for positive long-term survival outcomes 5
- There is an unmet medical need for early treatment of small melanocytic lesions or small melanomas in the eye to achieve local disease control and potentially prevent metastases 5