Kokemüller Lara, Ramachandran Dhanya, Schürmann Peter, Geffers Robert, Jentschke Matthias, Böhmer Gerd, Strauß Hans-Georg, Hirchenhain Christine, Schmidmayr Monika, Müller Florian, Fasching Peter A, Luyten Alexander, Häfner Norman, Hillemanns Peter, Dörk Thilo
Department of Gynaecology, Hannover Medical School, Hannover, Germany.
Genome Analytics, Helmholtz Centre for Infection Research, Braunschweig, Germany.
Int J Cancer. 2025 Feb 15;156(4):700-710. doi: 10.1002/ijc.35221. Epub 2024 Oct 23.
While cervical cancer is associated with a persistent human papillomavirus (HPV) infection, the progression to cancer is influenced by genomic risk factors that have remained largely obscure. Pathogenic variants in genes of the homology-directed repair (HDR) or mismatch repair (MMR) are known to predispose to diverse tumour entities including breast and ovarian cancer (HDR) or colon and endometrial cancer (MMR). We here investigate the spectrum of HDR and MMR germline variants in cervical cancer, with particular focus on the HPV status and histological subgroups. We performed targeted next-generation sequencing for 5 MMR genes and 12 HDR genes on 728 German patients with cervical dysplasia or invasive cancer. In total, 4% of our patients carried a pathogenic germline variant, based on ClinVar classifications and additional ESM1b and AlphaMissense predictions. These included 15 patients with truncating variants in HDR genes (BARD1, BRCA1, BRCA2, BRIP1, FANCM, RAD51D and SLX4). MMR-related gene variants were less prevalent and mainly of the missense type. While MMR-related gene variants tended to associate with adenocarcinomas, HDR gene variants were commonly observed in squamous cancers. While one patient with HPV-negative cancer carried a pathogenic MMR gene variant (in MSH6), the HDR germline variants were found in patients with HPV-positive cancers and tended to associate with HPV18. Taken together, our study supports a potentially risk-modifying role of MMR and HDR germline variants in cervical cancer but no association with HPV-negative status. These variants may be exploitable in future therapeutic managements.
虽然宫颈癌与人乳头瘤病毒(HPV)持续感染相关,但癌症进展受基因组风险因素影响,而这些因素在很大程度上仍不清楚。已知同源定向修复(HDR)或错配修复(MMR)基因中的致病变异易导致多种肿瘤实体,包括乳腺癌和卵巢癌(HDR)或结肠癌和子宫内膜癌(MMR)。我们在此研究宫颈癌中HDR和MMR种系变异的谱,特别关注HPV状态和组织学亚组。我们对728例德国宫颈发育异常或浸润性癌患者的5个MMR基因和12个HDR基因进行了靶向二代测序。根据ClinVar分类以及额外的ESM1b和AlphaMissense预测,我们的患者中共有4%携带致病变种系变异。其中包括15例HDR基因(BARD1、BRCA1、BRCA2、BRIP1、FANCM、RAD51D和SLX4)有截断变异的患者。与MMR相关的基因变异不太常见,主要为错义类型。虽然与MMR相关的基因变异倾向于与腺癌相关,但HDR基因变异常见于鳞状细胞癌。虽然1例HPV阴性癌症患者携带致病变异的MMR基因(在MSH6中),但HDR种系变异见于HPV阳性癌症患者,且倾向于与HPV18相关。综上所述,我们的研究支持MMR和HDR种系变异在宫颈癌中可能具有风险修饰作用,但与HPV阴性状态无关。这些变异可能在未来的治疗管理中得到应用。