Amelanocytic melanoma
ICD-10 C43.9 ICD-11 2C30.Z

First-Line Treatment of Unresectable Stage III/IV Amelanocytic Melanoma Without BRAF V600 Mutation

This protocol covers amelanocytic melanoma that has progressed to unresectable stage III or stage IV (distant metastasis) and where molecular testing has confirmed the absence of a BRAF V600 mutation (BRAF wild-type). This distinction directly shapes available first-line options and limits what is approved in subsequent lines.

Unresectable stage III or stage IV (metastatic) cutaneous melanoma confirmed as BRAF wild-type (BRAF V600 mutation absent). PD-1 blockade is a standard-of-care option in the first-line setting regardless of BRAF status; however, for BRAF wild-type disease, approved second-line options are notably limited — making the first-line choice especially consequential.

First-line management for this presentation involves immunotherapy, with the preferred regimen depending on whether a rapid response is clinically required and, for at least one option, on a specific tumour biomarker result. The full structured regimen — including agent selection criteria and sequencing — is available via the link below.

References

Based on these trials, PD-1 blockade is now an SoC option for all patients, regardless of tumour BRAF status, in the first-line setting.

For BRAF-WT melanoma, approved second-line options are very limited.

For patients with unresectable disease, first-line ipilimumab+nivolumab [ESMO-MCBS v1.1 score: A/4] is a preferred option for all patients regardless of BRAF status when this can be safely delivered for the first few months (i.e. when a rapid response is not required due to aggressive/symptomatic disease) [I, A].

First-line nivolumab [I, A; ESMO-MCBS v1.1 score: A/4] or pembrolizumab [I, A; ESMO-MCBS v1.1 score: A/4] is also recommended.

Nivolumab+relatlimab can be offered as first-line treatment but EMA approval is only for patients with tumour cell PD-L1 expression <1% [I, B; ESMO-MCBS v1.1 score: 3; EMA approved for PD-L1 expression <1%, FDA approval is regardless of PD-L1 expression].

DOI: 10.1016/j.annonc.2024.11.006 View source ↗