This Article Full Text Full Text (PDF) Purchase Article View Shopping Cart Alert me when this article is cited Alert me if a correction is posted Services Email this article to a colleague Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Save to my personal folders Download to citation manager Rights & Permissions Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Kolb, E. A. Articles by Meyers, P. A. Search for Related Content PubMed PubMed Citation Articles by Kolb, E. A. Articles by Meyers, P. A. Social Bookmarking                            What's this?

Long-Term Event-Free Survival After Intensive Chemotherapy for Ewing’s Family of Tumors in Children and Young Adults

From the Departments of Pediatrics, Surgery, Pathology, Biostatistics, and Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY.

Address reprint requests to Paul A. Meyers, MD, Department of Pediatrics, Memorial Sloan-Kettering Cancer Center, PO Box 139, 1275 York Ave, New York, NY 10021; e-mail: meyersp{at}mskcc.org.

Purpose: To improve the long-term event-free survival of patients with Ewing’s family of tumors (EFTs) using high-dose, short-term chemotherapy.

Patients and Methods: P6 was a prospective study of previously untreated patients with newly diagnosed EFTs. Patients received seven cycles of chemotherapy. Cycles 1, 2, 3, and 6 consisted of cyclophosphamide 2,100 mg/m²/d on days 1 and 2, and a 72-hour continuous infusion of doxorubicin 75 mg/m² and vincristine 2 mg/m² starting day 1. Cycles 4, 5, and 7 consisted of 5 consecutive days of ifosfamide 1,800 mg/m²/d and etoposide 100 mg/m²/d.

Results: Sixty-eight patients were enrolled from 1991 to 2001 (median age, 18.7 years; range, 3.7 to 39.9 years). At diagnosis, 44 patients had local-regional disease, and 24 had distant metastases. The 4-year event-free survival (EFS) rate for patients with local-regional disease is 82%; overall survival (OS) is 89%. The 4-year EFS rate for patients with distant metastases is 12%; the OS rate is 17.8%. All events occurred within 51 months of diagnosis. Four patients with distant metastases had progressive disease during therapy, and no patient with local-regional disease experienced disease progression during therapy.

Conclusion: Sustained EFS and OS can be achieved with intensive chemotherapy in children and young adults with local-regional EFTs. This therapy is relatively ineffective in the treatment of metastatic EFTs.

CiteULike    Complore    Connotea    Del.icio.us    Digg    Facebook    Reddit    Technorati    Twitter    What's this?

This article has been cited by other articles:

				S. Mani, D. Dutta, and B. K. De Primitive Neuroectodermal Tumor of the Liver: A Case Report Jpn. J. Clin. Oncol., December 7, 2009; (2009) hyp158v1. [Abstract] [Full Text] [PDF]

				M. S. Merchant and C. L. Mackall Current Approach to Pediatric Soft Tissue Sarcomas Oncologist, November 1, 2009; 14(11): 1139 - 1153. [Abstract] [Full Text] [PDF]

				K. Scotlandi, D. Remondini, G. Castellani, M. C. Manara, F. Nardi, L. Cantiani, M. Francesconi, M. Mercuri, A. M. Caccuri, M. Serra, et al. Overcoming Resistance to Conventional Drugs in Ewing Sarcoma and Identification of Molecular Predictors of Outcome J. Clin. Oncol., May 1, 2009; 27(13): 2209 - 2216. [Abstract] [Full Text] [PDF]

				K. Kikuta, N. Tochigi, T. Shimoda, H. Yabe, H. Morioka, Y. Toyama, A. Hosono, Y. Beppu, A. Kawai, S. Hirohashi, et al. Nucleophosmin as a Candidate Prognostic Biomarker of Ewing's Sarcoma Revealed by Proteomics Clin. Cancer Res., April 15, 2009; 15(8): 2885 - 2894. [Abstract] [Full Text] [PDF]

				S HARI, T P JAIN, S THULKAR, and S BAKHSHI Imaging features of peripheral primitive neuroectodermal tumours Br. J. Radiol., December 1, 2008; 81(972): 975 - 983. [Abstract] [Full Text] [PDF]

				M. Paulussen, A. W. Craft, I. Lewis, A. Hackshaw, C. Douglas, J. Dunst, A. Schuck, W. Winkelmann, G. Kohler, C. Poremba, et al. Results of the EICESS-92 Study: Two Randomized Trials of Ewing's Sarcoma Treatment--Cyclophosphamide Compared With Ifosfamide in Standard-Risk Patients and Assessment of Benefit of Etoposide Added to Standard Treatment in High-Risk Patients J. Clin. Oncol., September 20, 2008; 26(27): 4385 - 4393. [Abstract] [Full Text] [PDF]

				I. Y. Cheung, Y. Feng, K. Danis, N. Shukla, P. Meyers, M. Ladanyi, and N.-K. V. Cheung Novel Markers of Subclinical Disease for Ewing Family Tumors from Gene Expression Profiling Clin. Cancer Res., December 1, 2007; 13(23): 6978 - 6983. [Abstract] [Full Text] [PDF]

				M. E. Miller, L. Emerson, F. Clayton, B. G. Bentz, R. E. Data, K. L. Salzman, L. M. Smith, and M. K. Yu Extraosseous Ewing's Sarcoma J. Clin. Oncol., October 20, 2007; 25(30): 4845 - 4848. [Full Text] [PDF]

				R. K. Heck Jr., T. D. Peabody, and M. A. Simon Staging of Primary Malignancies of Bone CA Cancer J Clin, November 1, 2006; 56(6): 366 - 375. [Abstract] [Full Text] [PDF]

				O. Oberlin, A. Rey, A. S. Desfachelles, T. Philip, D. Plantaz, C. Schmitt, E. Plouvier, O. Lejars, H. Rubie, P. Terrier, et al. Impact of High-Dose Busulfan Plus Melphalan As Consolidation in Metastatic Ewing Tumors: A Study by the Societe Francaise des Cancers de l'Enfant J. Clin. Oncol., August 20, 2006; 24(24): 3997 - 4002. [Abstract] [Full Text] [PDF]

				M. Bernstein, H. Kovar, M. Paulussen, R. L. Randall, A. Schuck, L. A. Teot, and H. Juergensg Ewing's Sarcoma Family of Tumors: Current Management. Oncologist, May 1, 2006; 11(5): 503 - 519. [Abstract] [Full Text] [PDF]

				E. Hendershot Treatment Approaches for Metastatic Ewing's Sarcoma: A Review of the Literature Journal of Pediatric Oncology Nursing, November 1, 2005; 22(6): 339 - 352. [Abstract] [PDF]

				O. M. Tirado, S. Mateo-Lozano, and V. Notario Roscovitine Is an Effective Inducer of Apoptosis of Ewing's Sarcoma Family Tumor Cells In vitro and In vivo Cancer Res., October 15, 2005; 65(20): 9320 - 9327. [Abstract] [Full Text] [PDF]

				M. A. Clark, C. Fisher, I. Judson, and J. M. Thomas Soft-Tissue Sarcomas in Adults N. Engl. J. Med., August 18, 2005; 353(7): 701 - 711. [Full Text] [PDF]

				H.-Y. Huang, P. B. Illei, Z. Zhao, M. Mazumdar, A. G. Huvos, J. H. Healey, L. H. Wexler, R. Gorlick, P. Meyers, and M. Ladanyi Ewing Sarcomas With p53 Mutation or p16/p14ARF Homozygous Deletion: A Highly Lethal Subset Associated With Poor Chemoresponse J. Clin. Oncol., January 20, 2005; 23(3): 548 - 558. [Abstract] [Full Text] [PDF]