目的 总结伴有神经内分泌副瘤综合征的嗅神经母细胞瘤临床及病理特点。方法 回顾性分析2010-08至2020-08医院伴有神经内分泌副瘤综合征的4例嗅神经母细胞瘤患者临床及病理资料。其中男2例,女2例。年龄22～48岁, 平均39.5岁。根据改良Kadish分期,4例病变均为C期;根据Dulguerov分期,T3期1例,T4期3例。结果 所有患者均行内镜下肿瘤切除术,并获得阴性切缘。围术期3例出现尿崩、电解质紊乱及激素改变。术后1例出现颅内积血,无脑脊液漏等并发症。术后2周内尿崩、电解质紊乱及激素改变大致恢复正常。术后随访6～125个月,未见肿瘤复发及转移。免疫组化染色见肿瘤细胞中突触素(Syn)、嗜铬素A(ChgA)等多呈阳性,Ki-67染色显示细胞增殖指数为1%～50%。结论 嗅神经母细胞瘤患者伴发的神经内分泌症状隐匿,可表现为电解质及激素水平紊乱,围术期风险高,需提高对该病的认识。随访中定期监测电解质水平,可及时发现肿瘤复发。
Abstract
Objective To summarize the clinical diagnosis and treatment features of olfactory neuroblastoma(ONB) with paraneoplastic syndromes (PNS).Methods Four ONB patients with PNS in our department from August 2010 to August 2020 were reviewed. Their clinical data were collected. There were 2 males and 2 females, with an average age of 39.5 years. According to the modified Kadish stage, all the patients were Kadish stage C; while according to Dulguerov stage, there was 1 case of T3 and 3 cases of T4.Results All patients underwent endoscopic resection with negative margins. Diabetes insipidus, electrolyte disturbance and hormone changes occurred in 3 patients during the perioperative period. Postoperative complications such as cerebrospinal fluid leakage and meningitis were not found, but 1 patient had intracranial hematocele. Diabetic insipidus, ion disorders, and hormone changes generally returned to normal about 2 weeks after surgery. The follow-up time was 6-125 months, and no tumor recurrence or metastasis was observed. Hormone replacement therapy was not performed. Immunohistochemical staining showed that most of the tumor cells were positive for synaptophysin (Syn) and pheochromochrome A(ChgA), and Ki-67 staining showed cell proliferation index ranging from 1% to 50%.Conclusions ONB patients with PNS usually have insidious symptoms and need to be vigilant. These patients are prone to electrolyte disturbance and hormone level disturbance during the perioperative period. Electrolyte levels can be monitored regularly to prevent tumor recurrence.
关键词
嗅神经母细胞瘤 /
副瘤综合征 /
鼻内镜手术 /
抗利尿激素分泌不当综合征 /
异位ACTH综合征
Key words
olfactory neuroblastoma /
paraneoplastic syndromes /
endoscopic /
syndrome of inappropriate antidiuretic hormone /
ectopic ACTH secretion
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基金
国家自然科学基金(81770977);北京市科学技术委员会首都临床特色应用研究专项(Z181100001718103);首都卫生发展科研专项重点项目(首发2020-1-2051)
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