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.
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. 颅底头颈部肉瘤诊疗专家共识(2024版)[J]. 中华肿瘤杂志,2025,47(03):211-227.
DOI:10.3760/cma.j.cn112152-20240603-00235版权归中华医学会所有。
未经授权,不得转载、摘编本刊文章,不得使用本刊的版式设计。
除非特别声明,本刊刊出的所有文章不代表中华医学会和本刊编委会的观点。
一、骨肉瘤一线化疗用药推荐
甲氨蝶呤(M)、多柔比星(A)、顺铂(C)、异环磷酰胺(I),给药方式可考虑序贯用药或联合用药。参考的剂量范围为:甲氨蝶呤8~12 g/m 2,顺铂120~140 mg/m 2,多柔比星75~90 mg/m 2,异环磷酰胺12~15 g/m 2。以上为单药应用推荐剂量,若联合用药(如MAPI、MAP、AP等方案),则需酌情减量。
二、骨肉瘤二线化疗方案
IE方案:异环磷酰胺4 g/m 2 第1天,依托泊苷200 mg/m 2 第2~4天,每3~4周为1个治疗周期。
三、软组织肉瘤一线方案
1. VAC方案:长春新碱1.5 mg/m 2(最大单次剂量2 mg)+更生霉素1.5 mg/m 2(最大单次剂量2.5 mg)+环磷酰胺1.2~2.2 g/m 2。
VDC方案:长春新碱1.5 mg/m 2 (最大单次剂量2 mg) + 多柔比星75 mg/m 2(最大累积剂量375 mg/m 2)+环磷酰胺1.2~2.2 g/m 2。
2. VI方案: 长春新碱1.5 mg/m 2(最大单次剂量2 mg)+伊立替康 100 mg/m 2。
3. IE方案:异环磷酰胺9 g/m 2第1天,依托泊苷500 mg/m 2。
VAC和VI交替方案及VDC和IE交替方案可用于中高危软组织肉瘤化疗。
四、软组织肉瘤二线方案
1. 环磷酰胺+托泊替康方案:250 mg/m 2第1~5天,0.75 mg/m 2第1~5天,每3周为1个治疗周期。
2. 表柔比星+异环磷酰胺方案:表柔比星 60 mg/m 2第1~2天,异环磷酰胺7.5 g/m 2 第1~5天,每3周为1个治疗周期。
3. 安罗替尼方案:安罗替尼8~12 mg/d,第1~14天,每1周1个周期。
4. 瑞戈非尼方案:第1个周期可采用计量滴定的方法:即第1周80 mg/d,第2周 120 mg/d,第3周 160 mg/d。第2个周期开始推荐剂量为160 mg,第1~21天,每4周1个周期。
5. 帕博利珠单抗方案:帕博利珠单抗200 mg第1天,每3周1个周期。

你好,我可以帮助您更好的了解本文,请向我提问您关注的问题。