Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma.
OBJECTIVE
To examine the long-term effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma.


METHODS
From 1967 to 1994, a total of 30 children with head and neck rhabdomyosarcoma received megavoltage radiotherapy at one institution. Seventeen patients (57%) have survived and have at least a 5-year follow-up. There were 11 males and 6 females, with a median age of 5.7 years (range 2.2-11.6) at the time of radiotherapy. Tumor location was orbit in 6 patients, infratemporal fossa in 4, paranasal sinuses in 2, and supraglottic larynx in 2; the nasopharynx, pterygopalatine fossa, and parotid gland were sites for the remaining children. All but 2 patients had tumors of embryonal histology. The Intergroup Rhabdomyosarcoma Study (IRS) Group was I in 2, II in 3, and III in 11 children; 1 patient had a recurrent tumor after surgery alone. Radiotherapy volume was the primary tumor or tumor bed in 13, tumor and whole brain in 3, and tumor and craniospinal axis in 1. Median radiotherapy dose to the primary site was 5,040 cGy (range 4,140-6,500) and to the whole brain was 3,000 cGy. All but 1 were treated with 150-200-cGy fractions; 1 patient received 250-cGy fractions for a tumor in the larynx. Chemotherapy was vincristine (V), actinomycin-D (A), and cyclophosphamide (C) in 10 patients, VAC + adriamycin in 2, VA in 1, VA + ifosfamide in 1, VC + adriamycin in 1, and none in 2. One patient had salvage chemotherapy consisting of cisplatin and etoposide. Median follow-up time was 20 years (range 7.5-33).


RESULTS
Late effects of treatment were seen in all patients and included facial growth retardation in 11, neuroendocrine dysfunction in 9, visual/orbital problems in 9, dental abnormalities in 7, hearing loss in 6, and hypothyroidism in 3. Intellectual and academic delays were documented in 3 patients who had received whole brain radiotherapy. While neuroendocrine, thyroid, dental, and cognitive sequelae were primarily attributed to radiotherapy, hearing loss was thought to be a direct result of tumor destruction and, in 1 case, cisplatin chemotherapy. Late effects at or beyond 10 years from radiotherapy were few, but severe, and included chondronecrosis, esophageal stenosis, second malignancy, and brain hemorrhage.


CONCLUSIONS
Late effects of treatment in children receiving radiotherapy for head and neck rhabdomyosarcoma are frequent. Although radiotherapy is a significant contributor of neuroendocrine, dental, thyroid, and cognitive toxicity, it is not usually implicated with hearing loss. Late toxicity of treatment beyond 10 years is not as frequent as those occurring within 10 years of therapy.
Paulino
A C
AC
Department of Radiology, Division of Radiation Oncology, The University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA. arnold-paulino@uiowa.edu
Simon
J H
JH
Zhen
W
W
Wen
B C
BC
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D002648:Child; D002675:Child, Preschool; D003051:Cochlea / Q000187:drug effects / Q000528:radiation effects; D003071:Cognition / Q000528:radiation effects; D003131:Combined Modality Therapy; D016371:Cranial Irradiation / Q000009:adverse effects; D003817:Dentition; D004522:Educational Status; D005146:Facial Asymmetry / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006128:Growth / Q000528:radiation effects; D013006:Growth Hormone / Q000172:deficiency / Q000528:radiation effects; D006258:Head and Neck Neoplasms / Q000188:drug therapy / Q000532:radiotherapy*; D006801:Humans; D007031:Hypothalamus / Q000528:radiation effects; D008297:Male; D009918:Orbital Neoplasms / Q000532:radiotherapy; D010902:Pituitary Gland / Q000528:radiation effects; D011879:Radiotherapy Dosage; D011882:Radiotherapy, High-Energy / Q000009:adverse effects*; D012208:Rhabdomyosarcoma / Q000188:drug therapy / Q000532:radiotherapy*; D013997:Time Factors; D014786:Vision Disorders / Q000209:etiology
D013006:Growth Hormone