Orbital rhabdomyosarcoma: multidisciplinary treatment experience
1Department of Radiation Oncology, Royal Adelaide Hospital, Adelaide, South Australia, Australia. dion.forstner@sswahs.nsw.gov.au
Australasian Radiology
|February 28, 2006
Summary
Childhood orbital rhabdomyosarcoma (RMS) treatment shows high survival rates but significant late effects. Future strategies must balance cure rates with reducing long-term toxicities for better outcomes.
Area of Science:
- Pediatric Oncology
- Ophthalmology
- Radiation Oncology
Background:
- Orbital rhabdomyosarcoma (RMS) comprises 10% of childhood RMS cases.
- Current 5-year survival rates approach 85%, prompting focus on treatment sequelae.
- Multidisciplinary treatment is standard for orbital RMS.
Purpose of the Study:
- To review treatment outcomes for orbital RMS.
- To evaluate the incidence and nature of late effects following multidisciplinary treatment.
- To identify strategies for improving long-term patient outcomes.
Main Methods:
- Retrospective review of orbital RMS patients treated between 1982 and 2002.
- Inclusion criteria: multidisciplinary treatment including radiation and chemotherapy.
- Data collected on patient demographics, treatment, survival, and late effects.
Main Results:
- Five eligible patients (age 5.5-12 years) were identified.
- Significant late effects observed: facial bone hypoplasia, cataracts, growth hormone deficiency.
- Overall survival was 80% (4/5) with a mean follow-up of 8 years.
Conclusions:
- High survival rates in orbital RMS necessitate a focus on minimizing treatment-related late effects.
- Standardized toxicity assessment and long-term follow-up are crucial.
- Advanced radiation techniques (3D-CRT, IMRT, proton therapy) may reduce late effects and improve outcomes.

