Sarcomas of the head and neck in adult patients: current concepts and future perspectives

Abstract

Sarcomas comprise a heterogeneous and biologically diverse group of malignant neoplasms having as a common denominator their origin from mesenchymal cells. Head and neck sarcomas account for 4 to less than 20% of total body sarcomas depending on the criteria, such as age of patients (pediatric vs adult population), type of sarcomas (soft-tissue vs bony sarcomas) and site of location. Although head and neck sarcomas occur infrequently in adults, in the pediatric population one in three sarcomas will occur in the head and neck region. Most head and neck sarcomas are of the soft-tissue type, with only 20% being of bony or cartilaginous origin. Sarcomas display a diverse array of histologies and a wide spectrum of clinical behavior, ranging from relatively slow growing lesions to aggressive locally and regionally destructive tumors with the potential for systemic metastases. Osteosarcomas, rhabdomyosarcomas, pleomorphic sarcomas (malignant fibrous histiocytomas), fibrosarcomas and angiosarcomas are among the most common histologic types of sarcoma found in the head and neck. Surgery has been the primary therapeutic approach for the management of head and neck sarcomas. Survival rates for head and neck sarcomas suggest worse outcomes than for their extremity counterparts. Lymph node metastasis only occurs in 3-10% of sarcomas of the head and neck. An improvement in local disease control has recently been suggested with the combined use of surgery and radiotherapy. Conflicting results have been reported on the benefit from the use of chemotherapy as an adjuvant or neoadjuvant regimen, especially for high-grade sarcomas in long-term survival or local disease control. Encouraging results have recently been reported with the use of molecular targeted therapies with tyrosine kinase inhibitors and antiangiogenetic agents.