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Treatment of children with peripheral primitive neuroectodermal tumor or extraosseous Ewing's tumor with
S Gururangan1, N M Marina, X Luo
1Department of Hematology-Oncology, St. Jude Children's Research Hospital, University of Tennessee, Memphis, USA.
Insights
Peripheral primitive neuroectodermal tumor (PNET) and extraosseous Ewing's tumor (EOE) patients treated with Ewing's-directed therapy, including ifosfamide and etoposide, showed promising survival rates. This combined-modality approach achieved outcomes comparable to osseous Ewing's tumors.
Area of Science:
- Pediatric Oncology
- Medical Oncology
- Cancer Research
Background:
- Peripheral primitive neuroectodermal tumors (PNET) and extraosseous Ewing's tumors (EOE) are rare pediatric malignancies.
- Effective treatment strategies for these soft tissue tumors are crucial for improving patient outcomes.
Purpose of the Study:
- To evaluate the treatment and outcomes of pediatric patients with peripheral PNET/EOE using Ewing's-directed therapy.
- To assess the efficacy of an ifosfamide and etoposide 'window' regimen as part of induction therapy.
Main Methods:
- Seventeen pediatric patients with peripheral PNET or EOE received a 9-week ifosfamide and etoposide 'window' induction therapy.
- Subsequent treatment included cyclophosphamide and Adriamycin, surgery, radiotherapy, and maintenance chemotherapy for a total of 45 weeks.
Main Results:
- All 9 evaluable patients responded to the ifosfamide and etoposide window therapy (8 partial, 1 objective).
- At 49-94 months post-diagnosis, 12 patients were disease-free, with a 5-year overall survival of 77% and progression-free survival of 62%.
Conclusions:
- Combined-modality therapy directed at Ewing's tumors yields survival rates similar to osseous Ewing's tumors for patients with soft tissue PNET/EOE.
- The ifosfamide and etoposide combination demonstrates significant activity and should be included in future treatment protocols.
Purpose:
We report the treatment and outcome of patients with peripheral primitive neuroectodermal tumor (PNET) and extraosseous Ewing's tumor (EOE) using Ewing's-directed therapy, including an ifosfamide and etoposide window.
Methods:
Seventeen pediatric patients with peripheral PNET (n = 14) or EOE (n = 3) were enrolled between 1988 and 1992 on our institutional Ewing's protocol. Induction therapy comprised a 9-week "window" of ifosfamide and etoposide, followed by 9 weeks of therapy with cyclophosphamide and Adriamycin (Adria Laboratories, Columbus, OH). Response assessment after 17 weeks was followed by surgery and/or radiotherapy (doses based on tumor size and response to induction), repeat evaluation, and maintenance chemotherapy with alternating courses of vincristine/dactinomycin, ifosfamide/etoposide, and cyclophosphamide/Adriamycin for a total of 45 weeks.
Results:
At diagnosis, 8 patients had large lesions (>8 cm) and 3 had pulmonary metastases (1 with large tumor). Surgical resection was performed at diagnosis for 9 patients and after induction therapy for 5. During window therapy, all of the 9 evaluable patients responded (8 partial, I objective), and no patient without measurable disease developed disease progression. Responses were maintained or improved during subsequent induction in six of the patients with residual disease. Fourteen patients received local radiotherapy. At 49 to 94 months after diagnosis, 12 patients are disease-free (1 in second remission), 4 have died, and 1 is alive with disease. The five-year overall and progression-free survival rates are 77 +/- 13% and 62 +/- 16%, respectively.
Conclusion:
The use of consistent Ewing's-directed combined-modality therapy for patients with soft tissue peripheral PNET/EOE results in survival similar to that of patients with osseous Ewing's tumor. The combination of ifosfamide and etoposide appears active and should be incorporated in future treatment protocols.