Pediatric orofacial and laryngopharyngeal rhabdomyosarcoma. An Intergroup Rhabdomyosarcoma Study report.
Eighty-nine children with localized rhabdomyosarcoma of orofacial and laryngopharyngeal sites were treated in accordance with the first and second Intergroup Rhabdomyosarcoma Study (IRS) protocols (IRS-I and IRS-II) between 1972 and 1984. Treatment included surgery (or biopsy) and chemotherapy for all patients and radiotherapy in the majority. The actuarial estimate of the three-year survival rate for all patients was 83% and did not differ significantly by primary site, histologic findings, or presence of adenopathy. A trend for a worse survival rate was seen in clinical group III patients and in those less than 5 years of age at diagnosis. Factors associated with an increased risk of local/regional relapse included omission of radiotherapy and a radiation dose of less than 40 Gy (4000 rad). We conclude that treatment of these patients as recommended in the IRS-I and IRS-II protocols results in very good local and regional tumor control and survival rates. Salvage therapy for local/regional recurrence may yield long-term remission and possibly cure.
Wharam
M D
MD
Division of Radiation Oncology, Johns Hopkins Oncology Center, Baltimore, MD 21205.
Beltangady
M S
MS
Heyn
R M
RM
Lawrence
W
W
Raney
R B
RB
Ruymann
F B
FB
Soule
E H
EH
Tefft
M
M
Maurer
H M
HM
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D005153:Facial Neoplasms / Q000628:therapy; D005260:Female; D006258:Head and Neck Neoplasms / Q000401:mortality / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007822:Laryngeal Neoplasms / Q000628:therapy; D008297:Male; D009062:Mouth Neoplasms / Q000628:therapy; D010610:Pharyngeal Neoplasms / Q000628:therapy; D011446:Prospective Studies; D011897:Random Allocation; D012208:Rhabdomyosarcoma / Q000401:mortality / Q000628:therapy*