He Siyu, Xie Yuyao, Zhu Yinan, Wang Ziyue, Fu Lin, Lin Xuyong
Department of Pathology, The First Hospital of China Medical University, Shenyang, Liaoning, China.
Department of Gastroenterology, The First Hospital of China Medical University, Shenyang, Liaoning, China.
Virchows Arch. 2025 Jun 25. doi: 10.1007/s00428-025-04162-x.
Pulmonary papillary adenoma (PPA) constitutes a relatively uncommon type of lung neoplasm, which gives rise to a diagnostic intricacy. Owing to the paucity, our understanding of this disease remains far from comprehensive, and a thorough molecular investigation around PPA has been conspicuously lacking. The current study amassed a group of 10 cases of PPA that had been precisely diagnosed. We collected their clinical particulars and utilized next-generation sequencing (NGS) technology to examine the genetic mutations in 7 of these cases. The study population comprised 3 female and 7 male patients, with a median age of 52.9 years. The tumor sizes ranged from 0.8 to 3.8 cm. Seven patients were asymptomatic, while three patients manifested respiratory symptoms. Through the application of NGS, a wide array of mutation patterns was identified in the 7 cases, implicating pathways such as Wnt, RTK/RAS, PI3K, and Cell Cycle/Checkpoint. More precisely, mutations in genes such as MAP2K1 (1/7), AKT1 (1/7), NF1 (1/7), APC (2/7), EGFR (1/7), NBN (1/7), and CTNNB1 (1/7) were detected. The pathways most commonly involved were the Wnt pathway (3/7, APC and CTNNB1) and the RTK/RAS pathway (3/7, MAP2K1, NF1, and EGFR). As of now, all patients are still alive, without any evidence of recurrence or metastasis. This study has significantly enlarged the PPA case database and has suggested that PPA is a tumor type possessing unique and relatively complex molecular characteristics.
肺乳头状腺瘤(PPA)是一种相对罕见的肺肿瘤类型,这给诊断带来了复杂性。由于病例稀少,我们对这种疾病的了解仍远不全面,而且明显缺乏围绕PPA的深入分子研究。当前研究收集了一组10例已被精确诊断的PPA病例。我们收集了它们的临床细节,并利用二代测序(NGS)技术检测了其中7例的基因突变。研究人群包括3名女性和7名男性患者,中位年龄为52.9岁。肿瘤大小在0.8至3.8厘米之间。7名患者无症状,而3名患者表现出呼吸道症状。通过应用NGS,在7例病例中鉴定出了各种各样的突变模式,涉及Wnt、RTK/RAS、PI3K和细胞周期/检查点等信号通路。更确切地说,检测到了如MAP2K1(1/7)、AKT1(1/7)、NF1(1/7)、APC(2/7)、EGFR(1/7)、NBN(1/7)和CTNNB1(1/7)等基因的突变。最常涉及的信号通路是Wnt信号通路(3/7,APC和CTNNB1)和RTK/RAS信号通路(3/7,MAP2K1、NF1和EGFR)。截至目前,所有患者仍然存活,没有任何复发或转移的迹象。这项研究显著扩大了PPA病例数据库,并表明PPA是一种具有独特且相对复杂分子特征的肿瘤类型。