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Soft Tissue Tumors

Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue

Bone Marrow Transplantation volume 30, pages 297–302 (2002)Cite this article

Abstract

The MMT4 study was designed to explore an intensive chemotherapy regimen (MMT4–89) and the role of high-dose melphalan (MMT4–91) in children with metastatic soft tissue sarcoma, including extraosseous peripheral neuroectodermal tumor (PNET). Thirty-one patients with PNET were treated between 1989 and 1995 (11 according to MMT4–89 and 20 according to MMT4–91). Chemotherapy consisted of four CEVAIE cycles, each including three 3-week courses: CEV (carboplatin 500 mg/m2, epirubicin 150 mg/m2, vincristine 1.5 mg/m2), IVA ifosfamide 9 g/m2, actinomycin 1.5 mg/m2, vincristine 1.5 mg/m2), IVE (ifosfamide 9 g/m2, etoposide 600 mg/m2, vincristine 1.5 mg/m2). In MMT4–91 the fourth CEVAIE was replaced with melphalan 200 mg/m2 with stem cell rescue. The CEV combination was evaluated as a window study. Surgery followed the second cycle. Radiotherapy was administered to post-surgical residual disease. The response rate was 55% after CEV, rising to 80% after the first CEVAIE. Twenty-five patients achieved complete remission (CR). Overall, the 5-year EFS was 22.6%: 36.4% and 15% for patients treated according to MMT4–89 and MMT4–91, respectively (P = 0.3). Local control was achieved in 77% of irradiated patients vs 45% of non-irradiated. Age >10 years was associated with significantly poorer outcome (P = 0.04). In conclusion, despite the high CR rate, intensive chemotherapy with or without high-dose melphalan appeared to have little impact on the survival of patients with metastatic extraosseus PNET.

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Acknowledgements

The authors thank Ilaria Zanetti and Joan Malings for data managing and secretarial assistance. This research has been partially supported by a grant from the Fondazione Città della Speranza.

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Authors and Affiliations

  1. Hematology/Oncology Division, Department of Pediatrics, Padova, Italy

    G Bisogno, M Carli, R Colombatti & L De Zen

  2. The Birmingham Children's Hospital, Birmingham, UK

    M Stevens

  3. Department of Pediatrics, Institut Gustave Roussy, Villejuif, France

    O Oberlin

  4. Department of Oncology/Hematology, Olga Hospital, Stuttgart, Germany

    J Treuner

  5. Division of Radiotherapy, Hospital of Padova, Padova, Italy

    G Scarzello

  6. Children's Department, Royal Marsden NHS Trust, Sutton, UK

    CR Pinkerton

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  1. G Bisogno
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  2. M Carli
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Bisogno, G., Carli, M., Stevens, M. et al. Intensive chemotherapy for children and young adults with metastatic primitive neuroectodermal tumors of the soft tissue. Bone Marrow Transplant 30, 297–302 (2002). https://doi.org/10.1038/sj.bmt.1703617

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  • DOI: https://doi.org/10.1038/sj.bmt.1703617

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