Lenzi Jacopo, Salvati Maurizio, Frati Alessandro, Raco Antonino, Pichierri Angelo, Giangaspero Felice, Delfini Roberto
Department of Neurosurgery, University La Sapienza, Rome, Italy.
Childs Nerv Syst. 2006 Jan;22(1):95-8. doi: 10.1007/s00381-004-1092-2. Epub 2005 Mar 31.
Central neurocytoma is a tumor of neuronal origin that should be taken into consideration in the differential diagnosis of intraventricular neoplasms. Reports of neurocytomas with an extraventricular localization are rare: to our knowledge, the case described here is the first in which a neurocytoma developed within the ventricles but also invaded the brain stem.
The authors describe the unusual case of a 5-year-old boy with an intraventricular neurocytoma presenting with massive involvement of the basal nuclei and the brain stem. The patient underwent first biopsy and then surgery for ventricular-peritoneal shunting and partial removal of the tumor. Histology showed the tumor to be a typical neurocytoma with Mib-1 <2%. The postoperative course was uneventful. At 3 years' follow-up, the patient's clinical condition is stable and there are no signs of disease progression.
The literature is reviewed and the characteristics of this unusual tumor are discussed.
中枢神经细胞瘤是一种起源于神经元的肿瘤,在脑室肿瘤的鉴别诊断中应予以考虑。关于室管膜外定位的神经细胞瘤的报道很少:据我们所知,此处描述的病例是首例在脑室内发生但也侵犯脑干的神经细胞瘤。
作者描述了一名5岁男孩的罕见病例,该男孩患有脑室内神经细胞瘤,基底核和脑干大量受累。患者首先接受了活检,然后进行了脑室-腹腔分流术及肿瘤部分切除术。组织学检查显示肿瘤为典型的神经细胞瘤,Mib-1<2%。术后过程顺利。在3年的随访中,患者的临床状况稳定,没有疾病进展的迹象。
回顾了文献并讨论了这种罕见肿瘤的特征。