山东大学耳鼻喉眼学报 ›› 2022, Vol. 36 ›› Issue (3): 290-296.doi: 10.6040/j.issn.1673-3770.0.2022.009
• 临床研究 • 上一篇
陈家宏,王琳,姜彦,于龙刚,张继生,张志勇,孔静文
CHEN Jiahong, WANG Lin, JIANG Yan, YU Longgang, ZHANG Jisheng, ZHANG Zhiyong, KONG Jingwen
摘要: 目的 分析原发性鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤的发病特点,为该病的诊疗提供参考。 方法 回顾性分析2014年3月至2019年7月收治入院的2例鼻腔鼻窦骨外尤文肉瘤/外周原始神经外胚层肿瘤患者的临床资料,并检索2006年至2021年国内外相关文献,共48例(含本文2例)患者纳入研究,总结临床表现、治疗方法及预后。 结果 本组患者7~83岁、中位数26岁,男女比例1∶1.5,以鼻部症状为主,鼻塞16/28(57.1%)、鼻出血/涕中带血12/28(42.9%)、头痛3/28(10.7%)、嗅觉减退3/28(10.7%)。病变侵犯部位:鼻腔32/48(66.7%)、上颌窦24/48(50.0%)、筛窦27/48(56.3%)、蝶窦8/48(16.7%)、额窦3/48(6.3%),侵袭眼眶17/48(35.4%)、颅底6/48(12.5%)。单因素分析:综合治疗(P=0.009)、放疗(P=0.014)可明显改善患者预后,病变侵犯蝶窦(P=0.027)、颅底(P=0.002)提示患者预后不良,多因素COX回归分析示侵犯颅底为预后的独立危险因素(P=0.008),与未侵犯颅底的患者相比,侵犯颅底的患者死亡风险更高,HR=8.940(95%CI:1.756~45.506)。 结论 鼻腔鼻窦骨外尤文肉瘤(Extra-skeletal Ewing Sarcoma, EES)好发于青年女性,症状以鼻塞、鼻出血/涕中带血多见,病变范围主要累及鼻腔、上颌窦、筛窦,可侵及眼眶、颅底,病变侵犯颅底为死亡的独立危险因素,治疗以化疗联合放疗的综合治疗为主。
中图分类号:
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