Rare skin cancer and uveal melanoma

This cancer group is also dedicated to non-cutaneous melanoma such as uveal melanoma and mucosal melanoma, whose natural history, biology and management is in sharp contrast to skin melanomas. Multidisciplinarity is essential in the diagnostic and therapeutic phase, and also to generate innovative clinical and translational research programs. For example, in uveal melanoma (UM), immune checkpoint inhibitors show disappointing results when compared to cutaneous melanoma. Tebentafusp (Tebe) is a T-cell–redirecting bispecific fusion protein HLA A*02:01–restricted, using a high-affinity T-cell receptor targeting the glycoprotein 100, highly expressed on UM cells. Tebe is the first treatment to improve survival in a randomised phase III trial, and the first FDA-approved drug in advanced UM.
A randomised trial in the adjuvant setting is coming soon in patients with a high-risk of metastasis. Loco-regional treatments including surgery, radiofrequency ablation and liver-directed therapies are options in oligometastatic UM patients and should be discussed in multidisciplinary tumour boards. Patients should be referred to expert centres for dedicated clinical trials.

Progresses in survival will arise from the gathering of groups of experienced specialists in national and international networks, which may provide collaborative efforts to better understand the biology of rare skin cancers and non-cutaneous melanoma, and to develop innovative treatment approaches.