Sarcomas of the head and neck are also rare. Most commonly encountered sarcomas are those involving the mandible and maxilla as an osteosarcoma. Also seen within the oral cavity are lymphomas. Other tissue sarcomas seen in children and young adults are rhabdomyosarcoma.
Rhabdomyosarcoma is the most common sarcoma in children. Overall, rhabdomyosarcoma accounts for 8-20% of sarcomas in patients of all ages (92). Distribution of the tumor type depends on anatomic region; however, in children head and neck sarcomas account for 30-50% of these cases (92). There are four histologic subtypes of rhabdomyosarcoma: embryonal, alveolar, pleomorphic, and botryoid.
Cytogenetic typing and histologic subtyping has prognostic significance in rhabdomyosarcoma. Due to the histologic subtypes having significant impact on prognosis, tumor procurement for cytogenetic analysis and molecular investigation are paramount in patient treatment (93). Tissue biopsy for purposes of standard his-tologic examination should be submitted as well as fresh tissue for the purposes of ancillary molecular studies (see intra-operative consultation). Radical surgery for head and neck rhabdomyosarcoma has been largely replaced by the use of irradiation and chemotherapy. Surgical intervention has been used to excise small accessible tumors, reduce tumor bulk followed by other Multi-modality treatments or as a post-therapy resection (92).
Osteosarcomas of the head and neck are relatively infrequent. In a rather large study of osteosarcoma, approximately 6.5% of the cases involved skull, mandible, maxilla, facial bones, or cervical vertebrae (92). In the United States, osteosarcoma of head and neck accounts for approximately 6-13% of all osteosarcomas (94). Osteosarcoma of the long bones occurs most commonly in the second decade of life. In contrast, head and neck osteosarcomas typically occur in the third and fourth decades of life with equal gender distribution (95). Head and neck osteosarcomas are more problematic from a surgical standpoint due the anatomic constraints of obtaining negative surgical margins. Within the oral cavity, osteosarcoma of the mandible is a more favorable surgical site while the maxillary antrum is the most difficult site to manage surgically (94). Review of multi-modality studies has been addressed elsewhere (94).
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