Burdenko Neurosurgical Center, Moscow, Russia.
Central Clinical Hospital with Polyclinic of the Presidential Administration, Moscow, Russia.
Zh Vopr Neirokhir Im N N Burdenko. 2024;88(4):92-99. doi: 10.17116/neiro20248804192.
Dysembryoplastic neuroepithelial tumor (DNET) is a benign mixed neuronal-glial neoplasm (WHO grade 1). DNET is most often localized in temporal lobes and found in children and young people with epilepsy. There a few cases of DNET in ventricular system with dissemination along the ependyma in the world literature.
We present a rare case of T1- and T2-negative ventricular system tumor. Only FIESTA imaging revealed dissemination with multiple focal lesions of the third ventricle, its bottom and lateral walls, anterior horns of lateral ventricles, cerebellar vermis, cervical and lumbar spinal cord.
The patient underwent transcortical endoscopic biopsy of the third ventricle tumor with simultaneous ventriculoperitoneal shunting. DNET was diagnosed, and radiotherapy was subsequently performed. Literature data on this issue were analyzed.
To date, disseminated forms of DNET are extremely rare. X-ray features and morphological results allow us to establish the correct diagnosis and determine further treatment strategy.
胚胎发育不良性神经上皮肿瘤(DNET)是一种良性混合性神经元-神经胶质肿瘤(WHO 分级 1 级)。DNET 最常发生于颞叶,见于伴发癫痫的儿童和年轻人。世界文献中仅有少数脑室系统 DNET 病例,沿室管膜扩散。
我们报告了一例罕见的 T1 和 T2 阴性脑室系统肿瘤。只有 FIESTA 成像显示出沿第三脑室、其底部和侧壁、侧脑室前角、小脑蚓部、颈段和腰段脊髓的多个局灶性病变的扩散。
患者接受了第三脑室肿瘤经皮质内镜活检,同时进行了脑室-腹腔分流术。诊断为 DNET,随后进行了放疗。分析了该问题的文献数据。
迄今为止,DNET 的播散形式极为罕见。X 射线特征和形态学结果有助于确立正确的诊断,并确定进一步的治疗策略。