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Updated: Jan 23, 2026

A Mouse Model of Incompletely Resected Soft Tissue Sarcoma for Testing Neoadjuvant Therapies
Published on: July 28, 2020
Pazopanib maintenance therapy after tandem high-dose chemotherapy for disseminated Ewing sarcoma
Akihiro Tamura1, Nobuyuki Yamamoto1, Nanako Nino1
11Department of Hematology and Oncology, Kobe Children's Hospital, Minatojima-Minamimachi 1-6-7, Chuo-ku, Kobe, 650-0047 Japan.
Abstract:
The dismal prognosis of patients with disseminated Ewing sarcoma necessitates the development of novel treatment strategies. Pazopanib is an oral multi-targeted tyrosine kinase inhibitor that is active against advanced soft tissue sarcoma. However, the clinical activity and feasibility of pazopanib for treating Ewing sarcoma remain poorly understood. Moreover, clinical information on the use of tandem high-dose chemotherapy for Ewing sarcoma is limited. A 14-year-old boy with Ewing sarcoma was transferred to our hospital for treatment. Magnetic resonance imaging, computed tomography scans, and bone scintigraphy revealed multiple lesions in the pubis, ilium, ischium, femur, rib, cranial bone, thoracic vertebrae, sacrum, obturator muscle, adductor magnus muscle, testicular cord, and lungs. Bone scintigraphy after intensive chemotherapies confirmed that multiple abnormal accumulations were still present in the cranial bone and pubis. Subsequently, the patient received tandem high-dose chemotherapy including topotecan, and radiotherapy. Abnormal accumulations have disappeared in bone scintigraphy. Subsequently, pazopanib maintenance therapy was initiated. Despite the presence of innumerable lesions at diagnosis, the patient has been in near-complete remission for the past 1 year with pazopanib administration. This confirms that adding pazopanib maintenance therapy after tandem high-dose chemotherapy is a therapeutic option for cases with disseminated Ewing sarcoma.
Insights
Novel treatment combining pazopanib maintenance therapy with high-dose chemotherapy shows promise for disseminated Ewing sarcoma. This approach achieved near-complete remission in a young patient with widespread disease.
Area of Science:
- Pediatric Oncology
- Medical Oncology
- Pharmacology
Background:
- Disseminated Ewing sarcoma has a poor prognosis, necessitating innovative therapeutic strategies.
- Pazopanib, a tyrosine kinase inhibitor, shows activity in soft tissue sarcomas, but its role in Ewing sarcoma is unclear.
- Limited data exists on tandem high-dose chemotherapy for Ewing sarcoma treatment.
Observation:
- A 14-year-old patient with widely disseminated Ewing sarcoma presented with multiple bone and lung lesions.
- Initial intensive chemotherapies and radiotherapy reduced but did not eliminate disease.
- Pazopanib maintenance therapy was initiated after achieving remission with chemotherapy and radiotherapy.
Findings:
- Pazopanib maintenance therapy, following tandem high-dose chemotherapy and radiotherapy, led to near-complete remission in a patient with disseminated Ewing sarcoma.
- The patient remained in remission for one year on pazopanib maintenance.
- This case suggests pazopanib maintenance is a viable option for advanced Ewing sarcoma.
Implications:
- Adding pazopanib maintenance therapy to standard treatments may improve outcomes for disseminated Ewing sarcoma.
- This strategy warrants further investigation in clinical trials for pediatric sarcoma patients.
- Pazopanib offers a potential therapeutic option for refractory or metastatic Ewing sarcoma.
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