颅底头颈部肉瘤诊疗专家共识(2024版)  

The expert consensus on the diagnosis and treatment of skull base and head-neck sarcomas(2024 edition)

作　　者：中国抗癌协会肿瘤神经病学专业委员会 中国医学教育协会头颈肿瘤专业委员会 中国医药教育协会肿瘤化学治疗专业青年委员会 万经海 安常明 刘雨桃 蔡洪庆 慕逢春 方诚 王伟 何洁[5] 郭致飞[5] 张溪微 王朝阳 杨明 Chinese Anti-Cancer Association Cancer Neurology Committee;Chinese Medical Education Association Head and Neck Oncology Professional Committee;Chinese Medical Education Association Oncology Chemotherapy Youth Committee;Wan Jinghai;An Changming;Liu Yutao(不详;Department of Neurosurgery,National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100021,China;Department of Head and Neck Surgical Oncology,National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100021,China;Department of Medical Oncology,National Cancer Center/National Clinical Research Center for Cancer/Cancer Hospital,Chinese Academy of Medical Sciences and Peking Union Medical College,Beijing 100021,China)

机构地区：[1]不详 [2]国家癌症中心、国家肿瘤临床医学研究中心、中国医学科学院北京协和医学院肿瘤医院神经外科 [3]国家癌症中心、国家肿瘤临床医学研究中心、中国医学科学院北京协和医学院肿瘤医院头颈外科,北京100021 [4]国家癌症中心、国家肿瘤临床医学研究中心、中国医学科学院北京协和医学院肿瘤医院内科,北京100021 [5]安徽医科大学第二附属医院神经外科

出　　处：《中华肿瘤杂志》2025年第3期211-227,共17页Chinese Journal of Oncology

基　　金：中国医学科学院临床与转化医学研究(2022-I2M-C&T-B-063、2023-I2M-C&T-B-072);中国医学科学院医学与健康科技创新工程(2021-1-I2M-012);首都卫生发展科研专项项目(2024-2-40212);国家重点研发计划(2022YFC2705002);中国医学科学院肿瘤医院创新工程-医学健康领军科技人才养募专项(2024-12M-3-014);中国医学科学院肿瘤医院内科重点学科基金创新团队项目(CICASM-MOIT-202404)。

摘　　要：颅底头颈部肉瘤起源于颅底和头颈部的间质组织,具有高度侵袭性,可发生于所有年龄段。成人颅底头颈部肉瘤罕见,发病率不到头颈部肿瘤的1%,但在儿童人群中,颅底头颈部肉瘤发病率高达35%。头颈部肉瘤发病部位分布广泛,涉及颅底、面部和颈部的软组织、骨骼或神经。颅底头颈部肉瘤的病理类型多样,主要包括脂肪肉瘤、血管肉瘤、平滑肌肉瘤、横纹肌肉瘤、尤文肉瘤、骨肉瘤、未分化多形性肉瘤。治疗通常采用综合治疗策略,手术仍是首选治疗方案。但因颅底头颈部肉瘤手术难度大,放化疗疗效不一,整体治疗困难,目前中国尚无颅底头颈部肉瘤诊疗相关的指南与共识。为此,中国抗癌协会肿瘤神经病学专业委员会、中国医学教育协会头颈肿瘤专业委员会和中国医药教育协会肿瘤化学治疗专业青年委员会组织多学科专家进行研讨,基于循证医学证据,归纳出流行病学、组织及分子病理、影像学、手术、放疗、化疗、靶向治疗、免疫治疗等诊疗方面的临床应用推荐,旨在提供临床实践证据,提升颅底头颈部肉瘤的治疗水平,改善患者的预后和生活质量。Skull base sarcomas of the head and neck originate from the mesenchymal tissues of the skull base and head and neck region,exhibiting a highly aggressive behavior and can occur at all ages.Skull base sarcomas are rare in adults,with an incidence of less than 1%of head and neck tumors.However,in the pediatric population,the incidence can be as high as 35%.These tumors can arise in various locations,involving soft tissues,bones,or nerves of the skull base,face,and neck.The pathological types of skull base sarcomas are diverse,primarily including liposarcoma,angiosarcoma,leiomyosarcoma,rhabdomyosarcoma,Ewing sarcoma,osteosarcoma,undifferentiated pleomorphic sarcoma.Treatment typically involves a multimodal approach,with surgery being the preferred method.However,the surgical challenges posed by skull base sarcomas and the inconsistent efficacy of radiotherapy and chemotherapy contribute to the overall difficulty in treatment.Currently,there are no established guidelines or consensus for the diagnosis and management of skull base sarcomas in China.In response,Chinese Anti-Cancer Association Cancer Neurology Committee,Chinese Medical Education Association Head and Neck Oncology Professional Committee and Chinese Medical Education Association Oncology Chemotherapy Youth Committee have organized multidisciplinary expert discussions.Based on evidence from clinical research,we have summarized clinical application recommendations in the areas of epidemiology,histopathology,molecular pathology,imaging,surgery,radiotherapy,chemotherapy,targeted therapy,and immunotherapy,with the aim of providing clinical practice evidence to enhance the treatment of skull base sarcomas and improve patient prognosis and quality of life.

关 键 词：头颈部肿瘤 肉瘤 颅底 放疗 共识 

分 类 号：R73[医药卫生—肿瘤]