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Characteristics and Outcome of Patients With Refractory Orbital Rhabdomyosarcoma
Gianni Bisogno1,2, Maria Carmen Affinita2, Giuseppe Maria Milano3
1Department of Women's and Children's Health, University of Padua, Padua, Italy.
Background:
Orbital rhabdomyosarcoma (oRMS) is a rare pediatric soft-tissue sarcoma with generally excellent outcomes; however, a subset of patients relapse, and evidence on optimal salvage strategies and long-term survival after recurrence remains limited.
Methods:
Pediatric patients with oRMS enrolled in the cooperative protocols RMS79, RMS88, RMS96, and RMS2005 between 1979 and 2016.
Results:
Among 117 enrolled patients, 30 relapsed, predominantly with local recurrence. Relapse was associated with age < 1 year, female sex, RMS88 protocol, incomplete initial surgery, and omission of radiotherapy. Salvage treatments included multi-agent chemotherapy, radiotherapy, and surgery, with orbit exenteration performed in a total of 13 cases. After a median follow-up of 12.7 years from relapse, 10-year OS and EFS were 56.0% and 43.3%, respectively.
Conclusions:
Although oRMS usually has an excellent prognosis, relapse remains challenging. Multidisciplinary salvage therapy can yield long-term survival in over half of relapsed patients. Better initial treatment selection is needed to reduce relapse and late toxicity.
Insights
Orbital rhabdomyosarcoma (oRMS) relapse is challenging but survivable. Multidisciplinary salvage therapy offers long-term survival for over half of pediatric patients with recurrent oRMS.
Area of Science:
- Pediatric Oncology
- Sarcoma Research
- Clinical Outcomes
Background:
- Orbital rhabdomyosarcoma (oRMS) is a rare pediatric soft-tissue sarcoma with generally good outcomes.
- A subset of patients experience relapse, necessitating effective salvage strategies.
- Limited evidence exists on optimal treatment and long-term survival post-recurrence.
Purpose of the Study:
- To analyze outcomes and identify factors associated with relapse in pediatric orbital rhabdomyosarcoma.
- To evaluate the effectiveness of salvage therapies for recurrent oRMS.
- To inform strategies for reducing relapse and late toxicities.
Main Methods:
- Retrospective analysis of pediatric patients with oRMS.
- Data collected from cooperative protocols (RMS79, RMS88, RMS96, RMS2005) between 1979-2016.
- Evaluation of relapse risk factors and salvage treatment outcomes.
Main Results:
- 30 out of 117 patients relapsed, primarily with local recurrence.
- Relapse was linked to younger age, female sex, specific protocols, incomplete initial surgery, and lack of radiotherapy.
- Salvage options included chemotherapy, radiotherapy, and surgery; 13 patients underwent orbit exenteration.
- Median follow-up of 12.7 years post-relapse; 10-year overall survival (OS) was 56.0%, and event-free survival (EFS) was 43.3%.
Conclusions:
- Recurrence of oRMS presents a significant challenge despite generally favorable initial prognosis.
- Multidisciplinary salvage therapy demonstrates potential for achieving long-term survival in over half of relapsed cases.
- Improved initial treatment selection is crucial to minimize relapse rates and long-term side effects.
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