How is ciliary body melanoma treated?

Updated: Mar 03, 2020
  • Author: Enrique Garcia-Valenzuela, MD, PhD; Chief Editor: Andrew A Dahl, MD, FACS  more...
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Answer

Answer

The choice of management of ciliary body melanoma remains controversial in many respects. Although enucleation has been the treatment of choice in the past, the results of the COMS showed that vision-sparing approaches may offer similar degrees of ocular and metastasis tumor control. [6] At diagnosis, posterior uveal melanomas have already spread through micrometastasis in many patients.

Although undetected metastatic spread at the time of diagnosis and treatment of ciliary body melanoma is a major concern in every patient, adjuvant systemic treatment is not currently advocated. This consensus comes from treatment trials with intraocular melanomas and extrapolation of the experience with cutaneous melanoma, where adjuvant treatment has shown no benefit.

In cases in which distant metastases are found during the initial systemic workup, management of the intraocular melanomas becomes palliative. Systemic chemotherapy is the primary treatment. Many modalities and combinations of chemotherapeutic and immunotherapeutic agents are noted; however, for the most part, results continue to be disappointing. Increased survival times have been reported with high-dose interleukin-2 (IL-2) regimens, immunotherapy (eg, ipilimumab), inhibition of the mitogen-activated protein (MAP) kinase pathway (eg, with vemurafenib in patients whose tumors have a V600 mutation in the BRAF gene), glycoprotein peptide vaccine, anti–PD-1 and PD-L1 immune checkpoint inhibitors, trametinib (an orally available small-molecule selective inhibitor of MEK1 and MEK2), and the multikinase inhibitor. Although melanoma is a relatively radiation-resistant tumor, palliative radiation therapy may alleviate symptoms. This is an area of intense medical research with ever-increasing degrees of biologic sophistication being applied to new clinical trials.


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