Chuang Nathaniel A, Yoon Janet M, Newbury Robert O, Crawford John R
Departments of *Radiology †Pediatrics ‡Pathology §Neurosciences, San Diego and Rady Children's Hospital, University of California, San Diego, CA.
J Pediatr Hematol Oncol. 2014 Nov;36(8):e536-9. doi: 10.1097/MPH.0000000000000063.
Dysembryoplastic neuroepithelial tumors (DNETs) are considered as low-grade tumors commonly associated with intractable seizures. We report a case of an unusual hemispheric DNET in a young child presenting with new-onset focal seizures. The tumor was notable for its atypical neuroimaging features and very rapid malignant transformation into a glioblastoma multiforme in the absence of radiation or chemotherapy, 1-year postdiagnosis. Our case highlights the malignant potential of atypical DNETs in the absence of therapy.
胚胎发育不良性神经上皮肿瘤（DNETs）被认为是通常与难治性癫痫相关的低级别肿瘤。我们报告一例发生在幼儿的罕见半球型DNET病例，该患儿出现新发局灶性癫痫发作。该肿瘤以其非典型神经影像学特征以及在确诊后1年未经放疗或化疗的情况下迅速恶变成为多形性胶质母细胞瘤而引人注目。我们的病例突出了非典型DNETs在未接受治疗时的恶变潜能。
