Kalidindi Navya, Torres Carlos H, Michaud Jean, Zwicker Jocelyn Christine
Department of Neurology, University of Ottawa, The Ottawa Hospital, CHEO, Ottawa, Ont., Canada.
Department of Radiology, University of Ottawa, The Ottawa Hospital, CHEO, Ottawa, Ont., Canada.
Case Rep Oncol. 2014 Jul 16;7(2):471-7. doi: 10.1159/000365414. eCollection 2014 May.
Primitive neuroectodermal tumors (PNETs) are typically present as masses in children and adolescents, but rarely in adults. Diagnoses, management strategies, and prognostication factors are not well established in adult cases of PNETs. We describe the case of a central nervous system PNET diagnosed in a 55-year-old woman presenting with a sudden onset of symptoms consisting of increased intracranial pressure and findings of diffuse leptomeningeal enhancement and a small medullary lesion seen on MRI. Amongst the small database of PNETs diagnosed in adults, our case report stands out as one of few cases describing a primarily leptomeningeal PNET diagnosed on biopsy. We also review the literature on PNETs presenting with diffuse leptomeningeal disease and the treatment of PNETs in the adult population.
原始神经外胚层肿瘤(PNETs)通常表现为儿童和青少年的肿块,但在成人中很少见。成人PNETs病例的诊断、管理策略和预后因素尚未明确。我们描述了一例在一名55岁女性中诊断出的中枢神经系统PNET,该患者突然出现症状,包括颅内压升高,MRI显示弥漫性软脑膜强化和一个小的髓内病变。在已诊断的成人PNETs小数据库中,我们的病例报告是少数描述经活检诊断为主要软脑膜PNET的病例之一。我们还回顾了关于表现为弥漫性软脑膜疾病的PNETs以及成人PNETs治疗的文献。