Fiegl August, Hartmann Arndt, Junker Kerstin, Mink Jan, Stoehr Robert
Institut für Pathologie, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Krankenhausstraße 8-10, 91054, Erlangen, Deutschland.
Comprehensive Cancer Center EMN, Universitätsklinikum Erlangen, Friedrich-Alexander-Universität Erlangen-Nürnberg (FAU), Erlangen, Deutschland.
Pathologie (Heidelb). 2025 Feb;46(1):34-39. doi: 10.1007/s00292-024-01402-w. Epub 2024 Dec 18.
Penile carcinoma exhibits significant geographic variation in incidence, ranking 30th globally among newly diagnosed cancers with an annual rate of 0.84 cases per 100,000 men. Particularly high incidence rates of up to 2.2 are seen in Latin America, Asia, and Africa, largely due to a high prevalence of HPV, lower circumcision rates, and inadequate hygiene standards.The 2022 WHO classification of urogenital tumors continues to differentiate penile carcinomas based on their HPV status; however, the subdivision of numerous subtypes especially of the HPV(+) carcinomas was abandoned. This article aims to present current knowledge on the carcinogenesis of HPV(+) and HPV(-) penile carcinomas and their precursor lesions as well as updates from the latest WHO classification.Approximately 50% of penile carcinomas are caused by infection with high-risk HPV subtypes, with positive p16 immunohistochemistry serving as a good surrogate marker for HPV(+) tumors. HPV(-) carcinomas frequently show TP53 mutations and are associated with a poorer prognosis.While localized penile carcinomas have a relatively good prognosis, survival rates in metastatic cases remain poor. Neither microsatellite instability nor mismatch-repair deficiency appear to play a role, but up to 62.2% of tumors express PD-L1. Currently, immune checkpoint inhibitors such as Avelumab and Ipilimumab, along with antibody-drug conjugates targeting TROP2 and Nectin‑4, are being tested in clinical trials, potentially leading to the approval of targeted therapies for metastatic penile carcinoma in the future.
阴茎癌的发病率在全球范围内存在显著的地域差异,在新诊断的癌症中排名第30位,年发病率为每10万名男性0.84例。在拉丁美洲、亚洲和非洲,发病率特别高,可达2.2,这主要是由于人乳头瘤病毒(HPV)的高流行率、较低的包皮环切率和卫生标准不达标。2022年世界卫生组织(WHO)泌尿生殖系统肿瘤分类继续根据HPV状态对阴茎癌进行区分;然而,许多亚型的细分,尤其是HPV(+)癌的细分被摒弃。本文旨在介绍HPV(+)和HPV(-)阴茎癌及其前驱病变的致癌机制的当前知识,以及最新WHO分类的更新内容。大约50%的阴茎癌是由高危HPV亚型感染引起的,p16免疫组化阳性可作为HPV(+)肿瘤的良好替代标志物。HPV(-)癌经常显示TP53突变,且预后较差。虽然局限性阴茎癌的预后相对较好,但转移性病例的生存率仍然很低。微卫星不稳定性和错配修复缺陷似乎都不起作用,但高达62.2%的肿瘤表达程序性死亡受体1配体(PD-L1)。目前,阿维鲁单抗和伊匹木单抗等免疫检查点抑制剂,以及靶向滋养层细胞表面抗原2(TROP2)和脊髓灰质炎病毒受体相关蛋白4(Nectin-4)的抗体药物偶联物正在临床试验中进行测试,未来可能会批准针对转移性阴茎癌的靶向治疗。