Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, Whitefield, EPIP Area, Bangalore, 560066, Karnataka, India.
Department of Pathology, Sri Sathya Sai Institute of Higher Medical Sciences, Bangalore, 560066, Karnataka, India.
Childs Nerv Syst. 2024 Dec;40(12):4289-4294. doi: 10.1007/s00381-024-06578-3. Epub 2024 Aug 24.
Ganglioneuroblastoma (GNB) is a rare neurogenic tumor with a predilection for extracranial sites. Its primary intracranial occurrence is anecdotal, with less than ten cases reported in pediatric literature. We report two cases of this unusual entity. The first was a 1-year-old boy who presented with a progressive scalp swelling that radiologically appeared to be a mesenchymal tumor. The tumor was resected completely, and its histopathological examination was suggestive of a pigmented GNB. The clinico-radiological presentation and melanin pigmentation of the tumor were unique features of the case. The second case was a 7-year-old girl who presented with left hemiparesis and raised intracranial pressure due to a large right parietal intraparenchymal tumor that histologically proved to be a GNB. The child succumbed to disease progression a month after undergoing near-total resection of the tumor. Analysis of our cases in the light of a literature review reveals that pediatric intracranial GNBs have diverse clinico-radiological features and can easily be confused for commoner pathologies in both intra- and extra-axial locations.
神经节神经母细胞瘤(Ganglioneuroblastoma,GNB)是一种罕见的神经源性肿瘤,偏爱发生于颅外部位。其原发性颅内发生较为罕见,儿科文献中报道的病例不足 10 例。我们报告了两例这种不常见肿瘤的病例。第一例是一名 1 岁男孩,因进行性头皮肿胀就诊,影像学表现为间叶性肿瘤。肿瘤被完全切除,其组织病理学检查提示为色素性 GNB。该病例的临床-影像学表现和肿瘤的黑色素沉着是其独特特征。第二例是一名 7 岁女孩,因右侧顶叶脑实质内大肿瘤导致左侧偏瘫和颅内压升高就诊,该肿瘤的组织病理学检查证实为 GNB。患儿在接受肿瘤近全切除术后一个月因疾病进展而死亡。对我们病例的分析以及文献复习表明,儿童颅内 GNB 具有多样化的临床-影像学特征,在颅内和颅外部位均容易与更常见的病变混淆。