乳头状胶质神经元肿瘤的临床诊治

Clinical diagnosis and treatment of papillary glioneuronal tumor

目的:报告1例间变性乳头状胶质神经元肿瘤同时复习文献,归纳总结其诊断要点和治疗策略。方法:12岁患儿间断性头痛1个月就诊,MRI示左侧顶部及丘脑囊实性混杂信号肿块影,予以手术切除。术后病理证实Ki-67 Li约10%,属间变性乳头状胶质神经元肿瘤(WHOⅡ-Ⅲ)。结果:术后患者恢复满意,给予三维适形放射治疗(3D-CRT)57.6Gy/32f。放疗后1个月复查头颅MRI示左颞顶枕部颅骨及头皮软组织呈术后改变,局部未见明显异常对比增强病灶。结论:乳头状胶质神经元肿瘤大多表现良性,肿瘤全切后无复发、转移;增殖指数可能是判断PGNT预后的关键指标,Ki-67较高者术后应行辅助治疗。

Objective：To report one case of anaplastic papillary glioneuronal tumor （PGNT） and review the literature. Methods： A 12 -year- old boy had a intermittent headache for 1 month. MRI of his brain demonstrated a solid and cystic mass in the left parietal. The patient underwent a gross tumor resection. The pathological results confirmed a anaplastic papillary glioneuronal tumor. Results： The patient recovered well after operation and was given three dimentional conformal radiation therapy（3D -CRT）. Conclusion： Most of the currently recognized PGNT have a benign course. There are few aggressive examples described in the literature. The proliferative index might prove to be a key tool in providing prognostic information. PGNT with higher proliferative indices should be given postoperative adjuvant therapy.