Bone sarcomas of the head and neck in children - The St Jude Children's Research Hospital experience

BACKGROUND. Bone sarcomas of the head and neck are difficult to resect. The authors reviewed their institutional experience with these tumors to chara cterize patients' clinical findings and to assess the impact of surgical re section on outcome. METHODS. The records of the 28 patients with bone sarcomas originating in t he head and neck treated at St Jude Children's Research Hospital between Ma rch 1962 and January 1998 were reviewed. RESULTS. There were 10 males and 18 females (median age, 12.6 years) each w ith a single sarcoma: osteosarcoma (18), Ewing sarcoma (7), malignant fibro us histiocytoma (MFH) (2), and fibrosarcoma (1). Primary tumor sites includ ed the maxilla (13), skull (10), mandible (2), and other sites (3). All but one patient with Ewing sarcoma had localized disease at the time of diagno sis. All patients underwent surgery: complete resection, 8; gross total res ection, 4; incomplete resection, 14; and biopsy only, 2; 22 also received c hemotherapy. Radiotherapy was given to all patients with Ewing sarcoma and to four patients with primary osteosarcoma. Twelve patients survived a medi an of 8.4 years after diagnosis, 14 died of disease, and 2 died of unrelate d causes. Local disease progression was evident in 12 patients (9 with oste osarcoma, 2 with MFH, and 1 with Ewing sarcoma) who died of disease, 9 of w hom had the initial treatment of biopsy alone or incomplete resection. Pati ents with osteosarcoma who had the initial treatment of incomplete resectio n or biopsy alone were more likely to experience local failure (P = 0.001) and had poorer survival (P = 0.014) than those who underwent complete or gr oss total resection. CONCLUSIONS. Bone sarcomas of the head and neck are rare among children and most often are localized at the time of diagnosis. Incomplete resection of osteosarcoma is associated with local failure and poor outcome. Although a ggressive surgery is essential for the cure of osteosarcoma, its necessity in the treatment of Ewing sarcomas remains controversial. (C) 2000 American Cancer Society.