Introduction: Dysembryoplastic neuroepithelial tumor (DNT) is a rare benign brain tumor predominantly involving children and young adults. Histologically, it corresponds to WHO Grade I tumors; however, it may masquerade aggressive neural tumors such as oligodendroglioma, oligoastrocytoma, pilocytic astrocytoma, and ganglioglioma. The literature on clinical, radiological, and pathological spectrum of DNT is described mostly in the form of case reports, with only a few case series reported till date.
Methods: A retrospective review of files with diagnosis of DNT (2016 to 2018) was made in the Department of Pathology, National Institute of Pathology, New Delhi. A total of ten cases were retrieved, and their clinical, radiological, and histopathological features were reviewed and studied. Special stains and immunohistochemistry were done, wherever required.
Results: The mean age was 14.8 (±7.9) years, with a male-to-female ratio of 1.5:1. The most common mode of presentation was recurrent, intractable seizures. The most common site of lesion was parietal lobe followed by temporal and frontal lobes of the brain. On histology, mucoid matrix admixed with floating neurons and oligodendrocyte-like cells was a consistent feature; however, the presence of specific glioneuronal elements was observed in only a few cases.
Conclusions: DNT is a benign, low-grade, nonrecurrent neuroepithelial neoplasm. It is important to differentiate this rare entity from other mimickers, as it is surgically curable and carries an excellent prognosis without the need for adjuvant chemotherapy and radiotherapy. The study helps to enrich the clinicopathological aspects of this rare but important entity.


中文翻译：

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胚胎发育不良性神经上皮肿瘤的临床和组织病理学特征：来自三级护理中心的经验


简介：胚胎发育不良性神经上皮瘤（DNT）是一种罕见的良性脑肿瘤，主要累及儿童和青年。组织学上，它对应于WHO I级肿瘤；然而，它可能会伪装成侵袭性神经肿瘤，如少突胶质细胞瘤、少突星形细胞瘤、毛细胞星形细胞瘤和神经节胶质瘤。关于 DNT 临床、放射学和病理学谱的文献大多以病例报告的形式进行描述，迄今为止只有少数病例系列报道。
方法：在新德里国家病理学研究所病理学系对诊断为 DNT 的文件（2016 年至 2018 年）进行了回顾性审查。共检索到 10 例病例，对其临床、影像学和组织病理学特征进行了回顾和研究。如有需要，可进行特殊染色和免疫组化。
结果：平均年龄为 14.8 (±7.9) 岁，男女比例为 1.5:1。最常见的表现方式是反复发作的顽固性癫痫发作。最常见的病变部位是顶叶，其次是大脑的颞叶和额叶。在组织学上，黏液基质混合有漂浮的神经元和少突胶质细胞样细胞是一致的特征；然而，仅在少数情况下观察到特定胶质神经元元素的存在。
结论： DNT是一种良性、低级别、非复发性神经上皮肿瘤。将这种罕见的实体与其他模仿者区分开来很重要，因为它可以通过手术治愈并且预后良好，无需辅助化疗和放疗。该研究有助于丰富这个罕见但重要的实体的临床病理学方面。