Sturgis Erich M, Potter Bryan O
Department of Head and Neck Surgery, The University of Texas M D Anderson Cancer Center, 1515 Holcombe Boulevard, Unit 441, Houston, TX 77030-4009, USA.
Curr Opin Oncol. 2003 May;15(3):239-52. doi: 10.1097/00001622-200305000-00011.
This review discusses the classification, etiology, diagnosis, evaluation, treatment, and prognosis of sarcoma of the head and neck region.
Sarcomas account for less than 1% of all malignancies in the United States with only 5 to 15% of these sarcomas occurring in the head and neck region. However, about 1 in 3 pediatric sarcomas will occur in the head and neck region. Occasionally, these tumors are associated with genetic syndromes or previous radiation exposures, but, most commonly, no clear etiology exists. Pathologic classification is critical to the ultimate treatment and prognosis of sarcoma of the head and neck. Osteosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, and angiosarcoma are the most common types of sarcoma to occur in the head and neck region; however, up to 20% of head and neck sarcomas will remain unclassified. Surgery has been central to the management of these malignancies with some exceptions in the pediatric population. Adjuvant chemotherapy is being utilized and/or studied for most high-grade sarcomas and adjuvant radiotherapy is important for disease control in high-grade soft-tissue sarcomas. Prognosis is clearly related to tumor grade and margin status.
Sarcomas of the head and neck region are rare malignancies often without a clear etiology. Expert pathologic review and classification is critical, as are quality imaging and multidisciplinary management.
本综述讨论头颈部肉瘤的分类、病因、诊断、评估、治疗及预后。
在美国，肉瘤占所有恶性肿瘤的比例不到1%，其中仅5%至15%发生于头颈部。然而，约三分之一的儿童肉瘤发生在头颈部。这些肿瘤偶尔与遗传综合征或既往放疗史有关，但最常见的是病因不明。病理分类对头颈部肉瘤的最终治疗及预后至关重要。骨肉瘤、横纹肌肉瘤、恶性纤维组织细胞瘤及血管肉瘤是头颈部最常见的肉瘤类型；然而，高达20%的头颈部肉瘤仍无法分类。手术一直是这些恶性肿瘤治疗的核心，但儿科患者有一些例外情况。大多数高级别肉瘤正在使用和/或研究辅助化疗，辅助放疗对高级别软组织肉瘤的疾病控制很重要。预后显然与肿瘤分级和切缘状态有关。
头颈部肉瘤是罕见的恶性肿瘤，通常病因不明。专家病理检查和分类至关重要，高质量成像和多学科管理也同样重要。
