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Localized paediatric orbital rhabdomyosarcoma: influence of imaging on treatment
B J Burns1, K McHugh, H P McDowell
1Radiology Department, John Radcliffe Hospital, Oxford, UK. bburns@doctors.org.uk
Insights
This review of pediatric orbital rhabdomyosarcoma found imaging varied across UK centers. Radiotherapy for residual masses at day 80 post-diagnosis is crucial for treatment outcomes.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Radiology
Background:
- Orbital rhabdomyosarcoma is the most common pediatric malignant orbital tumor.
- It generally presents with a favorable prognosis.
Purpose of the Study:
- To review imaging and treatment of localized orbital rhabdomyosarcoma in UK children.
- To assess adherence to treatment protocols and radiotherapy timing.
Main Methods:
- Retrospective review of 16 patients from the UK Children's Cancer Study Group (UKCCSG) database.
- Analysis of imaging and radiological reports based on the Malignant Mesenchymal Tumour '89 (MMT89) protocol.
- Assessment of radiotherapy administration and protocol adherence.
Main Results:
- Imaging techniques and reporting lacked standardization across centers.
- Tumor measurements were not consistently reported.
- Treatment protocols were not always strictly followed, particularly regarding residual masses at day 80.
- Fifteen of sixteen patients received radiotherapy.
Conclusions:
- Standardization of orbital imaging in pediatric rhabdomyosarcoma is lacking.
- Radiology reports should clearly state the presence or absence of post-therapeutic residual disease.
- Further research is needed to distinguish between post-treatment fibrosis and tumor recurrence.
- Timely radiotherapy for residual masses is likely more critical than imaging standardization.
Aim:
Orbital rhabdomyosarcoma is the most common primary malignant orbital tumour in children and has a good prognosis. The purpose of this paper was to review the imaging and consequent treatment of patients with localized orbital rhabdomyosarcoma from around the U.K.
Materials And Methods:
Patients were identified through the U.K. Children's Cancer Study Group (UKCCSG) database. Investigations and therapy were dictated by the Malignant Mesenchymal Tumour '89 (MMT89) protocol. Imaging and radiological reports of 16 patients from 12 centres were reviewed. The number of patients receiving radiotherapy, timing of radiotherapy, and adherence to treatment protocols were assessed.
Results:
Local radiologists' reports and imaging techniques varied between sequential examinations and centres. The imaging was adequate for management. No reports quoted measurements of the tumours. Treatment protocols were not always followed rigidly with regard to a residual mass at day 80 post-diagnosis. However, the protocol was not explicit for all outcomes. Fifteen out of 16 patients eventually received radiotherapy.
Conclusion:
There is no standardization of imaging between centres. The presence or absence of a post-therapeutic residue should be stated in the radiology report. Further investigation is needed to differentiate between fibrosis and recurrent tumour. Radiotherapy for residual mass at day 80 is probably more important than standardizing radiological technique.
