Not currently affiliated with any institution or company, Winston-Salem, NC, 27104, USA.
Fam Cancer. 2024 Nov;23(4):469-471. doi: 10.1007/s10689-024-00418-8. Epub 2024 Sep 5.
Non-small cell lung cancer is the most common cause of cancer death globally. When apparent incidental pathogenic germline variants (PGVs) are uncovered with routine next generation sequencing (NGS) of NSCLCs, germline testing (GT) is recommended to confirm that PGV. Because it is far more common that an uncovered tumor TP53 variant is related to a somatic event than an incidental PGV, however, GT for Li Fraumeni syndrome (LFS) is not recommended based solely on uncovering a NSCLC TP53 variant. Because nearly all tumor EGFR variants are also somatic in origin, GT is not recommended based solely on uncovering a tumor EGFR variant.However, there is evidence that patients with coexisting NSCLC variants in both EGFR and TP53 have significant likelihoods of having LFS. For patients with LFS, there are recommended measures for prevention and early detection of LFS-associated cancers and cascade GT of relatives for LFS. Although co-existing genetic variants in NSCLC are not currently used as a biomarker for GT to identify patients with PGVs, given the evidence reviewed here, select patients with NSCLCs that harbor this dual biomarker (i.e., co-existing TP53/EGFR variants) might reasonably be considered for GT for LFS.
非小细胞肺癌是全球癌症死亡的最常见原因。当在非小细胞肺癌的常规下一代测序(NGS)中发现明显偶然的种系致病性变异(PGV)时,建议进行种系检测(GT)以确认 PGV。然而,由于未发现的肿瘤 TP53 变异与体细胞事件相关的可能性远远大于偶然的 PGV,因此仅基于发现 NSCLC TP53 变异不推荐进行 Li Fraumeni 综合征(LFS)的 GT。由于几乎所有肿瘤 EGFR 变异也都是体细胞起源的,因此仅基于发现肿瘤 EGFR 变异不推荐进行 GT。然而,有证据表明同时存在 EGFR 和 TP53 共存的非小细胞肺癌变异的患者有很大可能患有 LFS。对于患有 LFS 的患者,有建议的预防和早期检测 LFS 相关癌症的措施,并对 LFS 相关亲属进行 GT 检测。尽管非小细胞肺癌中的共存遗传变异目前不作为 GT 的生物标志物来识别具有 PGV 的患者,但鉴于这里审查的证据,对于同时携带这种双重生物标志物(即共存的 TP53/EGFR 变异)的非小细胞肺癌患者,可以合理地考虑进行 LFS 的 GT。