Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • Original Article
  • Published:

Pediatric Transplants

Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor

Bone Marrow Transplantation volume 45, pages 907–911 (2010)Cite this article

Abstract

The RMS4.99 study was designed to explore the role of early sequential intensified chemotherapy (SICT) with PBSC rescue in patients with soft tissue sarcoma with a poor prognosis. Fourteen patients with desmoplastic small round-cell tumor (DSRCT) were included in this study. Initial chemotherapy was followed by a course of CY and etoposide with subsequent PBSC harvest, then three consecutive intensified chemotherapy combinations followed by PBSC rescue and G-CSF administration: first cycle thiotepa (150 mg/m2 × 2 on day 1) and melphalan (60 mg/m2 on day 2), second cycle CY (2 g/m2 on days 1 and 2) and thiotepa (150 mg/m2 × 2 on day 3), third cycle melphalan (80 mg/m2 on day 1). The interval between cycles had to be kept as short as possible. Then patients underwent surgery or radiotherapy or both, after which six courses of vincristine, actinomycin D, CY were administered. Ten patients received SICT, which was well tolerated. With a median follow-up of 27 months only three patients are alive without evidence of disease. The 3-year event-free and overall survival rates were 15.5 and 38.9%, respectively. The prognosis for pediatric patients with DSRCT did not improve after administering intensified chemotherapy early in their treatment, so different strategies are needed.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1

Similar content being viewed by others

References

  1. Gerald WL, Ladanyi M, de Alava E, Cuatrecasas M, Kushner BH, LaQuaglia MP et al. Clinical, pathologic, and molecular spectrum of tumors associated with t(11;22)(p13;q12): desmoplastic small round-cell tumor and its variants. J Clin Oncol 1998; 16: 3028–3036.

    Article  CAS  PubMed  Google Scholar 

  2. Kushner BH, LaQuaglia MP, Wollner N, Meyers PA, Lindsley KL, Ghavimi F et al. Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy. J Clin Oncol 1996; 14: 1526–1531.

    Article  CAS  PubMed  Google Scholar 

  3. Bisogno G, Roganovich J, Sotti G, Ninfo V, di Montezemolo LC, Donfrancesco A et al. Desmoplastic small round cell tumour in children and adolescents. Med Pediatr Oncol 2000; 34: 338–342.

    Article  CAS  PubMed  Google Scholar 

  4. Gerald WL, Rosai J . Desmoplastic small cell tumor with divergent differentiation. Pediatr Pathol 1989; 9: 177–183.

    Article  CAS  PubMed  Google Scholar 

  5. Hassan I, Shyyan R, Donohue JH, Edmonson JH, Gunderson LL, Moir CR et al. Intraabdominal desmoplastic small round cell tumors: a diagnostic and therapeutic challenge. Cancer 2005; 104: 1264–1270.

    Article  PubMed  Google Scholar 

  6. Saab R, Khoury JD, Krasin M, Davidoff AM, Navid F . Desmoplastic small round cell tumor in childhood: the St Jude Children's Research Hospital experience. Pediatr Blood Cancer 2007; 49: 274–279.

    Article  PubMed  Google Scholar 

  7. Bertuzzi A, Castagna L, Quagliuolo V, Ginanni V, Compasso S, Magagnoli M et al. Prospective study of high-dose chemotherapy and autologous peripheral stem cell transplantation in adult patients with advanced desmoplastic small round-cell tumour. Br J Cancer 2003; 89: 1159–1161.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Fraser CJ, Weigel BJ, Perentesis JP, Dusenbery KE, DeFor TE, Baker KS et al. Autologous stem cell transplantation for high-risk Ewing's sarcoma and other pediatric solid tumors. Bone Marrow Transplant 2006; 37: 175–181.

    Article  CAS  Google Scholar 

  9. Carli M, Colombatti R, Oberlin O, Stevens M, Masiero L, Frascella E et al. High-dose melphalan with autologous stem-cell rescue in metastatic rhabdomyosarcoma. J Clin Oncol 1999; 17: 2796–2803.

    Article  CAS  PubMed  Google Scholar 

  10. Bisogno G, Ferrari A, Bergeron C, Scagnellato A, Prete A, Alaggio R et al. The IVADo regimen—a pilot study with ifosfamide, vincristine, actinomycin D, and doxorubicin in children with metastatic soft tissue sarcoma: a pilot study on behalf of the European pediatric Soft tissue sarcoma Study Group. Cancer 2005; 103: 1719–1724.

    Article  CAS  PubMed  Google Scholar 

  11. Livaditi E, Mavridis G, Soutis M, Papndreu E, Moschovi M, Papadakis V et al. Diffuse intraabdominal desmoplastic small round cell tumor: a ten-year experience. Eur J Pediatr Surg 2006; 16: 423–427.

    Article  CAS  PubMed  Google Scholar 

  12. Al Balushi Z, Bulduc S, Mulleur C, Lallier M . Desmoplastic small round cell tumor in children: a new therapeutic approach. J Pediatr Surg 2009; 44: 949–952.

    Article  PubMed  Google Scholar 

  13. Lal DR, Su WY, Wolden SL, Loh KC, Modak S, La Quaglia MP . Results of multimodal treatment for desmoplastic small round cell tumors. J Pediatr Surg 2005; 40: 251–255.

    Article  PubMed  Google Scholar 

  14. Mazuryk M, Paterson AH, Temple W, Arthur K, Crabtree T, Stewart DA . Benefit of aggressive multimodality therapy with autologous stem cell support for intra-abdominal desmoplastic small round cell tumor. Bone Marrow Transplant 1998; 21: 961–963.

    Article  CAS  PubMed  Google Scholar 

  15. Roganovich J, Bisogno G, Cecchetto G, D'Amore ES, Carli M . Paratesticular desmoplastic small round cell tumor: case report and review of the literature. J Surg Oncol 1999; 71: 269–272.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

We thank Angela Scagnellato for her continuous support in data processing and Gloria Tridello for the statistical analysis. This research was partially supported by a grant from the Fondazione Città della Speranza.

Author information

Authors and Affiliations

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

    G Bisogno, A Rosolen & M Carli

  2. Pediatric Oncology Unit, Istituto Nazionale dei Tumori, Milano, Italy

    A Ferrari

  3. Department of Pathology, University Hospital of Padova, Padova, Italy

    R Alaggio

  4. Radiotherapy Division, Istituto Oncologico Veneto, Padova, Italy

    G Scarzello

  5. Division of Pediatric Oncology, Pediatric Hospital ‘G Gaslini’, Genova, Italy

    A Garaventa

  6. Pediatric Unit, Bari Hospital, Bari, Italy

    G Arcamone

Authors
  1. G Bisogno
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A Ferrari
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. A Rosolen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. R Alaggio
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. G Scarzello
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. A Garaventa
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. G Arcamone
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. M Carli
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to G Bisogno.

Ethics declarations

Competing interests

The authors declare no conflict of interest.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Bisogno, G., Ferrari, A., Rosolen, A. et al. Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor. Bone Marrow Transplant 45, 907–911 (2010). https://doi.org/10.1038/bmt.2009.248

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/bmt.2009.248

Keywords

This article is cited by

Search

Advanced search

Quick links