目的:在腺样囊性癌(ACC)的高级别转化(HGT)的罕见中,这项研究对其侵袭性和临床意义提供了前所未有的见解.
方法:从412例ACC病例中提取23例HGT患者与非HGT患者的1:1匹配比较,重点解剖独特的临床病理特征和预后结果。
结果:HGT的主要部位是鼻窦和泪腺(各30.4%)。值得注意的是,固体亚型是HGT中最普遍的模式,占病例的69.6%。与非HGT相比,HGT队列表现出明显更高的淋巴结转移率(39.1%vs.8.7%;P<0.05),神经周浸润(60.9%vs.26.1%;P<0.05),并增加Ki-67增殖指数(35.0%vs.10.0%;P<0.05)。此外,HGT区通常显示p63表达减少或缺失,以及高级病理形态学。HGT与复发增加(55.0%)和远处转移(78.3%)相关,平均生存期为35.9个月,3年死亡率为35.0%。HGT组的总体生存率和无进展生存率显着降低。
结论:这项研究代表了我们所知的最大的单中心HGT病例队列,强调其在鼻窦和泪腺中的频繁发生,并与较差的预后相关。研究结果支持将ACC中的HGT分类为4级,反映了其严重程度。
OBJECTIVE: Amidst the rarity of High-grade transformation (HGT) in adenoid cystic carcinoma (ACC), this study offers unprecedented insights into its aggressive nature and clinical implications.
METHODS: A 1:1 match comparison between 23 HGT patients and non-HGT counterparts was extracted from 412 ACC cases, focusing on dissecting distinctive clinicopathological features and prognostic outcomes.
RESULTS: The predominant sites of HGT were the sinonasal and lacrimal glands (30.4% each). Notably, the solid subtype was the most prevalent pattern within HGT, accounting for 69.6% of cases. Compared to non-HGT, the HGT cohort exhibited significantly higher rates of lymph node metastasis (39.1% vs. 8.7%; P < 0.05), perineural invasion (60.9% vs. 26.1%; P < 0.05), and increased Ki-67 proliferation index (35.0% vs. 10.0%; P < 0.05). Moreover, HGT regions typically showed reduced or absent p63 expression, along with high-grade pathomorphology. HGT was associated with increased recurrence (55.0%) and distant metastasis (78.3%), leading to an average survival of 35.9 months and a 3-years mortality rate of 35.0%. Overall and progression-free survival rates were significantly decreased in the HGT group.
CONCLUSIONS: This study represents the largest single-center cohort of HGT cases to our knowledge, highlighting its frequent occurrence in the sinonasal and lacrimal glands and association with poorer outcomes. The findings support classifying HGT in ACC as Grade 4, reflecting its severity.