Sarcomas of the head and neck region

Purpose of review This review discusses the classification, etiology, diagnosis, evaluation, treatment, and prognosis of sarcoma of the head and neck region. Recent findings 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. Summary 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.