目的:研究原始神经外胚叶肿瘤的临床及病理特点。方法:总结6例原始神经外胚叶肿瘤患者的临床表现、影像学表现、病理特点及治疗结果。6例患者均行手术治疗,其中2例急诊手术,4例常规手术,术后4例行全脑放疗,2例联合化疗。结果:显微镜下全切2例,2例颅鼻沟通瘤颅内病灶全切,部分切除2例。随访4～18个月,死亡1例,3例无病情进展生存,2例病情进展生存。结论:原始神经外胚叶肿瘤是一种恶性程度极高但对治疗较敏感的肿瘤。 Objective: To study the clinical and pathological features of primitive neuroectodermal tumors. Methods: The clinical manifestations, imaging findings, pathological features and treatment outcome of 6 patients with primitive neuroectodermal tumor were summarized. Six patients underwent surgery, including 2 emergency operations and 4 routine operations. Whole-brain radiotherapy was performed in 4 patients and combined chemotherapy in 2 patients. Results: 2 cases under the microscope, 2 cases of intracranial cranio-nasal tumor full cut, partial resection in 2 cases. After 4 to 18 months of follow-up, 1 patient died, 3 patients survived without progression and 2 patients survived. Conclusion: The primitive neuroectodermal tumor is a highly malignant tumor that is more sensitive to treatment.