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Long-term effects in children treated with radiotherapy for head and neck rhabdomyosarcoma
A C Paulino1, J H Simon, W Zhen
1Department of Radiology, Division of Radiation Oncology, The University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA. arnold-paulino@uiowa.edu
Insights
Long-term radiotherapy for pediatric head and neck rhabdomyosarcoma frequently causes late effects. While radiation contributes to neuroendocrine, dental, and cognitive issues, hearing loss is often tumor or chemotherapy-related.
Area of Science:
- Pediatric Oncology
- Radiation Oncology
- Medical Physics
Background:
- Rhabdomyosarcoma is a rare soft tissue sarcoma in children.
- Head and neck rhabdomyosarcomas require multimodal treatment, often including radiotherapy.
- Long-term outcomes and treatment-related toxicities in survivors are critical areas of study.
Purpose of the Study:
- To evaluate the long-term effects of megavoltage radiotherapy in children treated for head and neck rhabdomyosarcoma.
- To identify the types and frequency of late toxicities experienced by these patients.
- To determine the relationship between radiotherapy and specific late adverse events.
Main Methods:
- Retrospective analysis of 30 children with head and neck rhabdomyosarcoma treated with radiotherapy between 1967 and 1994.
- Detailed review of patient demographics, tumor characteristics, treatment details (radiotherapy dose, volume, chemotherapy), and long-term follow-up data.
- Assessment of late effects including facial growth, neuroendocrine function, vision, hearing, dental health, and cognitive function.
Main Results:
- All 17 long-term survivors (median follow-up 20 years) experienced late effects.
- Common toxicities included facial growth retardation (11), neuroendocrine dysfunction (9), visual/orbital problems (9), dental abnormalities (7), and hearing loss (6).
- Intellectual and academic delays were noted in 3 patients receiving whole brain radiotherapy; severe late effects beyond 10 years were rare but included chondronecrosis and second malignancy.
Conclusions:
- Late treatment effects are common in children with head and neck rhabdomyosarcoma following radiotherapy.
- Radiotherapy is a significant cause of neuroendocrine, dental, and cognitive toxicity.
- Hearing loss is more often attributed to tumor or chemotherapy than radiotherapy, and severe late toxicities beyond 10 years are infrequent.
Purpose:
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.
Conclusion:
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.