From the Department of Radiology (C.C.-V., F.G.G., L.O.T.-G., A.V.), Children's Hospital of Philadelphia, Philadelphia, Pennsylvania
From the Department of Radiology (C.C.-V., F.G.G., L.O.T.-G., A.V.), Children's Hospital of Philadelphia, Philadelphia, Pennsylvania.
AJNR Am J Neuroradiol. 2024 Apr 8;45(4):483-487. doi: 10.3174/ajnr.A8145.
Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) are rare brain tumors first described in 2017 and recently included in the 2021 5th World Health Organization Classification of Tumors of the Central Nervous System. They typically affect children and young adults. Few pediatric cases have been reported in the literature. The most common imaging features described, include location within the temporal lobe, involvement of the cortical/subcortical region, coarse calcifications, and well-defined margins with solid and cystic morphology, with slight-or-no enhancement. However, there is limited information on imaging features in children. We present the imaging spectrum of neuroimaging features in a series of pediatric patients with a histologically and molecularly proved PLNTY diagnosis. Coarse calcifications are uncommon in children compared with the adult literature, and they may develop with time. The transmantle-like sign can be observed, and adjacent cortical dysplasia may be seen. Seizure recurrence may occur despite gross total resection of the tumor.
年轻人多形性低级别神经上皮肿瘤(PLNTY)是一种罕见的脑肿瘤,于 2017 年首次描述,最近被纳入 2021 年第 5 版世界卫生组织中枢神经系统肿瘤分类。它们通常影响儿童和年轻成年人。文献中很少有儿科病例报道。最常见的影像学特征包括位于颞叶内、皮质/皮质下区域受累、粗钙化、边界清晰、实性和囊性形态、轻度或无增强。然而,关于儿童的影像学特征的信息有限。我们展示了一系列经组织学和分子证实为 PLNTY 诊断的儿科患者的神经影像学特征的影像学谱。与成人文献相比,儿童的粗钙化并不常见,并且可能随着时间的推移而出现。可以观察到脑膜样征,并且可能存在相邻皮质发育不良。尽管肿瘤完全切除,但癫痫仍可能复发。