Dozza Diego Cassol, Rodrigues Flávio Freinkel, Chimelli Leila
Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro RJ, Brazil.
Arq Neuropsiquiatr. 2012 Sep;70(9):710-4. doi: 10.1590/s0004-282x2012000900012.
Dysembryoplastic neuroepithelial tumor (DNT), described in 1988 and introduced in the WHO classification in 1993, affects predominantly children or young adults causing intractable complex partial seizures. Since it is benign and treated with surgical resection, its recognition is important. It has similarities with low-grade gliomas and gangliogliomas, which may recur and become malignant.
To investigate whether DNT was previously diagnosed as astrocytoma, oligodendroglioma, or ganglioglioma and to determine its frequency in a series of low-grade glial/glio-neuronal tumors.
Clinical, radiological, and histological aspects of 58 tumors operated from 1978 to 2008, classified as astrocytomas (32, including 8 pilocytic), oligodendrogliomas (12), gangliogliomas (7), and DNT (7), were reviewed.
Four new DNT, one operated before 1993, previously classified as astrocytoma (3) and oligodendroglioma (1), were identified. One DNT diagnosed in 2002 was classified once more as angiocentric glioma. Therefore, 10 DNT (17.2%) were identified.
Clinical-radiological and histopathological correlations have contributed to diagnose the DNT.
胚胎发育不良性神经上皮肿瘤（DNT）于1988年被描述，并于1993年被引入世界卫生组织分类，主要影响儿童或年轻人，可导致难治性复杂部分性癫痫发作。由于其为良性且通过手术切除治疗，因此对其进行识别很重要。它与低级别胶质瘤和神经节胶质瘤有相似之处，后者可能复发并恶变。
调查DNT是否曾被误诊为星形细胞瘤、少突胶质细胞瘤或神经节胶质瘤，并确定其在一系列低级别神经胶质/神经胶质-神经元肿瘤中的发生率。
回顾了1978年至2008年手术切除的58例肿瘤的临床、放射学和组织学特征，这些肿瘤被分类为星形细胞瘤（32例，包括8例毛细胞型）、少突胶质细胞瘤（12例）、神经节胶质瘤（7例）和DNT（7例）。
发现4例新的DNT，其中1例在1993年之前手术，之前被分类为星形细胞瘤（3例）和少突胶质细胞瘤（1例）。2002年诊断的1例DNT再次被分类为血管中心性胶质瘤。因此，共识别出10例DNT（17.2%）。
临床-放射学和组织病理学相关性有助于DNT诊断的确立。
