成人鞍区非典型畸胎样/横纹肌样瘤一例  

Adult sellar region atypical teratoid/rhabdoid tumors:a case report

作　　者：张义彪[1] 徐敬斌[1] 高亚峰[1] 常奎[1] 王鑫 张龙 成金民 ZHANG Yibiao;XU Jingbin;GAO Yafeng(Department of Neurosurgery,Second People's Hospital of Fuyang City,Fuyang 236015,China)

机构地区：[1]阜阳市第二人民医院神经外科,阜阳236015

出　　处：《临床神经外科杂志》2023年第5期594-596,600,共4页Journal of Clinical Neurosurgery

摘　　要：目的总结成人鞍区非典型畸胎样/横纹肌样瘤(AT/RT)的临床及病理特征,提高该病的诊断和治疗水平。方法回顾性分析阜阳市第二人民医院神经外科2019年7月收治的1例成年鞍区占位的手术及治疗过程。结果行经鼻蝶显微镜下全切除肿瘤,术后第4天出现头痛症状,经CT检查证实肿瘤在术后1周内复发且肿瘤体积明显大于术前。术后病理报告显示肿瘤Ki-67(+),高达80%,仅提示鞍区恶性肿瘤;在术后第24天做出AT/RT(成人变异性)、INI-1缺失的病理诊断。结论成人鞍区AT/RT为神经外科罕见疾病,临床及影像检查均无特异性,具有恶性程度高、术后生存期短等特点,目前尚未引起国内神经外科同道及病理学专家重视。Objective To summarize the clinical and pathological features of atypical teratoid/rhabdomyoid tumors(AT/RT)in adult sellar region,and to improve the diagnosis and treatment of the disease.Methods The operation and treatment of a patient with sellar space occupying lesion admitted to Department of Neurosurgery,Second People's Hospital of Fuyang City in July 2019 was analyzed retrospectively.Results The tumor was completely removed under a nasal transsphenoidal microscopic resection.The patient developed headache symptoms again on the 4th day after the surgery.CT examination confirmed that the tumor recurred within one week after the surgery and the tumor volume was significantly larger than that before the surgery.The postoperative pathology showed a tumor Ki-67(+),as high as 80%,indicating only malignant tumors in the sellar region.A pathological diagnosis of AT/RT(adult variability)and INI-1 deficiency was made on the 24th day after surgery.Conclusions The AT/RT in adult sellar region are rare diseases in neurosurgery.The clinical and imaging examinations are not specific.They have high malignant degree and short postoperative survival time.At present,it is not under concern of domestic neurosurgery colleagues and pathology experts.

关 键 词：非典型畸胎样/横纹肌样瘤 鞍区 KI-67 INI-1 

分 类 号：R739.41[医药卫生—肿瘤]