Clinical characteristics of neuroendocrine carcinoma of nose and skull base and analysis of curative effect of endoscopic surgery
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摘要: 目的 探讨鼻及颅底神经内分泌癌的临床特征及内镜下手术切除的疗效与预后。方法 回顾性分析7例确诊为鼻及颅底神经内分泌癌并行内镜下手术治疗的患者资料。7例均为小细胞神经内分泌癌，参照国际TNM分期：Ⅰ期1例，Ⅱ期1例，Ⅲ期1例，ⅣA期1例，ⅣB期3例。治疗方法均采用内镜下手术切除，其中术后放疗1例，化疗1例，放化疗3例。结果 随访9~58个月，2例复发后死亡，1例同侧颈部淋巴结转移(行颈淋巴结清扫术后无瘤生存)，4例在最后一次随访中均为无瘤生存。结论 鼻及颅底神经内分泌癌目前并无标准治疗方案，经内镜手术彻底切除肿瘤为主的综合性治疗是有效可行的治疗方案。Abstract: Objective To investigate the clinical features of neuroendocrine carcinoma of nose and skull base and the efficacy and prognosis of endoscopic resection.Methods The clinical data of 7 patients with neuroendocrine carcinoma of nose and skull base treated by endoscopic surgery were retrospectively analyzed. According to the international TNM staging, there were 1 case of stage Ⅰ, 1 case of stage Ⅱ, 1 case of stage Ⅲ, 1 case of stage ⅣA and 3 cases of stage ⅣB. All patients were treated by endoscopic surgery, including 1 case of postoperative radiotherapy, 1 case of chemotherapy and 3 cases of radiotherapy and chemotherapy.Results During the follow-up of 9-58 months, 2 cases died after recurrence, 1 case had ipsilateral cervical lymph node metastasis(tumor-free survival after neck dissection), and 4 cases had tumor-free survival at the last follow-up.Conclusion At present, there is no standard treatment for neuroendocrine carcinoma of the nose and skull base. The comprehensive treatment based on complete resection of the tumor by endoscopic surgery is an effective and feasible treatment.
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表 1 7例鼻及颅底NEC患者临床资料
例序 性别 年龄/岁 首发症状 主要病变部位 分期 治疗方式 随访时间/月 结局 1 男 47 右鼻出血 右鼻腔、右筛窦、前颅底 ⅣA期 内镜下颅底肿瘤切除+放化疗 57 术后2年复发，再次手术+术后放化疗，3年后死亡 2 男 73 左鼻塞、左鼻出血 左鼻腔、左上颌窦、筛窦、蝶窦及额窦、硬脑膜及左侧嗅球 ⅣB期 单纯内镜下颅底肿瘤切除 9 术后8个月复发，放弃治疗后1个月死亡 3 男 37 右鼻塞伴嗅觉下降 右鼻腔、右侧筛窦 Ⅱ期 内镜下颅底肿瘤切除+放疗 58 无瘤生存 4 男 38 双鼻塞、双耳听力下降 鼻咽部 Ⅰ期 内镜下颅底肿瘤切除+放化疗 37 无瘤生存 5 男 60 右侧鼻塞伴头痛 右鼻腔、右筛窦、前颅底、硬脑膜、右嗅球及嗅束 ⅣB期 单纯内镜下颅底肿瘤切除 21 术后7个月同侧颈部淋巴结转移，行颈淋巴结清扫后无瘤生存 6 男 54 右涕中带血 右侧鼻腔、右上颌骨、右上颌窦 Ⅲ期 内镜下颅底肿瘤切除+放化疗 14 无瘤生存 7 男 60 右侧鼻塞伴嗅觉下降 右鼻腔、右筛窦、蝶窦、硬脑膜累及右嗅球、嗅束 ⅣB期 内镜下颅底肿瘤切除+化疗 15 无瘤生存 
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表 2 7例鼻及颅底NEC患者免疫组织化学检查结果
例序 CgA Syn CEA S-100 CK CD56 Ki67/% 1 + + + - 点状+ - 80 2 + + + - + + 60 3 + + - - + 未查 30 4 + + 未查 - - 未查 20 5 + + - - + - 60 6 + + + + + + 未查 7 点状+ - + - 点状+ 区域+ 50
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