Nasri S, Mark R J, Sercarz J A, Tran L M, Sadeghi S
Division of Head and Neck Surgery, UCLA School of Medicine, CA, USA.
Am J Otolaryngol. 1995 May-Jun;16(3):165-71. doi: 10.1016/0196-0709(95)90096-9.
The treatment of children with rhabdomyosarcoma (RMS) has been established in multicenter protocols. There are few data available regarding the treatment of other sarcomas in pediatric patients.
From 1955 to 1988, a retrospective review of 33 children with sarcomas other than RMS of the head and neck was performed to identify prognostic factors and to determine optimal treatment. Grade, tumor size, and RMS grouping of the tumors were found to be of prognostic value.
Eighteen of 33 (54%) patients are currently disease free. Only 3 of 16 (19%) patients treated initially with surgery alone were rendered disease free. However, 6 patients were salvaged with an additional surgical procedure, yielding 9 of 16 (56%) patients disease free treated with surgery alone. Of patients receiving surgery plus radiation therapy, with or without chemotherapy, 7 of 14 (50%) have no evidence of disease.
After salvage therapy, 5-year disease-free survival was 56% (15 of 27). Unlike rhabdomyosarcoma, complete surgical extirpation is the mainstay of treatment in pediatric sarcomas other than RMS of the head and neck. Adjuvant radiation with or without chemotherapy should be administered when complete resection is not possible.