To advance our understanding of pulmonary ASC, we present a novel case of advanced ASC harboring concurrent driver mutations (KRAS G12C, BRAF (non-V600E), PIK3CA, FLT1) that demonstrated an exceptional response to immunotherapy, with progression-free survival (PFS) exceeding 46.5 months. This evidence concerns the gene BRAF and Tako-tsubo cardiomyopathy.