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

Abstract

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.