Wang Xinyao, Yin Qiufeng, Liu Shijie, Zhao Heng, Zhang Haibo
Department of Pediatric Neurosurgery, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China.
Department of Radiology, Xinhua Hospital Affiliated to Shanghai Jiao Tong University School of Medicine, Shanghai, 200092, China.
Childs Nerv Syst. 2025 Jul 1;41(1):223. doi: 10.1007/s00381-025-06880-8.
This study reports three cases of pediatric patients with polymorphous low-grade neuroepithelial tumor (PLNTY), confirmed by surgical pathology at our medical center, and presents their clinical and imaging features. PLNTY is a newly defined subtype in the 2021 WHO Classification of Tumors of the Central Nervous System (5th edition), categorized under pediatric-type diffuse low-grade glioma, WHO grade 1. The patients included one male and two females, aged 6, 9, and 9 years, respectively. The tumors were located in the thalamus, temporal lobe, and frontal lobe. All cases showed calcifications on CT scans. Magnetic resonance imaging (MRI) revealed low signal on T1-weighted images (T1WI) and high signal on T2-weighted images (T2WI), with localized enhancement after contrast administration. Diffusion-weighted imaging (DWI) demonstrated high signal in two cases and low signal in one case. Positron emission tomography-computed tomography (PET-CT) showed increased metabolic activity in two cases, raising suspicion of high-grade malignant brain tumors preoperatively. Postoperatively, none of the patients experienced tumor recurrence during follow-up periods ranging from 1 month to 6 years. This study highlights the clinical and imaging characteristics of PLNTY in pediatric patients and underscores the diagnostic challenges as well as the generally favorable postoperative prognosis.
本研究报告了3例经我院医学中心手术病理确诊的小儿多形性低度神经上皮肿瘤（PLNTY）病例，并介绍了其临床和影像学特征。PLNTY是2021年世界卫生组织中枢神经系统肿瘤分类（第5版）中新定义的亚型，归类于小儿型弥漫性低度胶质瘤，WHO 1级。患者包括1名男性和2名女性，年龄分别为6岁、9岁和9岁。肿瘤分别位于丘脑、颞叶和额叶。所有病例CT扫描均显示钙化。磁共振成像（MRI）显示T1加权像（T1WI）呈低信号，T2加权像（T2WI）呈高信号，增强后有局部强化。弥散加权成像（DWI）显示2例呈高信号，1例呈低信号。正电子发射断层扫描-计算机断层扫描（PET-CT）显示2例代谢活性增加，术前怀疑为高级别恶性脑肿瘤。术后，在1个月至6年的随访期内，所有患者均未出现肿瘤复发。本研究突出了小儿PLNTY的临床和影像学特征，强调了诊断挑战以及总体良好的术后预后。
