Department of Pathology, ICMR- National Institute of Pathology, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
Department of Neurosurgery, Vardhman Mahavir Medical College and Safdarjung Hospital, New Delhi, India.
J Cancer Res Ther. 2021 Jul-Sep;17(4):912-916. doi: 10.4103/jcrt.JCRT_632_19.
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.
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.
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.
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.
胚胎发育不良性神经上皮肿瘤（DNT）是一种罕见的良性脑肿瘤，主要发生在儿童和年轻成人中。组织学上，它对应于 WHO 分级 I 肿瘤；然而，它可能伪装为侵袭性神经肿瘤，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细胞瘤。关于 DNT 的临床、放射学和病理学谱的文献主要以病例报告的形式描述，迄今为止仅报道了少数病例系列。
在新德里国家病理学研究所病理科对诊断为 DNT（2016 年至 2018 年）的文件进行了回顾性审查。共检索到 10 例，回顾和研究了其临床、放射学和组织病理学特征。在需要时进行了特殊染色和免疫组织化学检查。
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复发性、难治性癫痫。最常见的病变部位是顶叶，其次是颞叶和额叶。组织学上，黏液基质混合漂浮神经元和少突胶质样细胞是一致的特征；然而，仅在少数情况下观察到特定的神经胶质细胞。
DNT 是一种良性、低级别、非复发性神经上皮肿瘤。将这种罕见实体与其他类似物区分开来非常重要，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一罕见但重要实体的临床病理方面。
