Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Multicenter Study
. 2011 Mar;47(5):724-31.
doi: 10.1016/j.ejca.2010.11.013. Epub 2010 Dec 8.

Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups

Andrea Ferrari  1 , Rosalba Miceli, Annie Rey, Odile Oberlin, Daniel Orbach, Bernadette Brennan, Luigi Mariani, Modesto Carli, Gianni Bisogno, Giovanni Cecchetto, Gian Luca De Salvo, Michela Casanova, Max M Vannoesel, Anna Kelsey, Michael C Stevens, Meenakshi Devidas, Alberto S Pappo, Sheri L Spunt
Affiliations
Multicenter Study

Non-metastatic unresected paediatric non-rhabdomyosarcoma soft tissue sarcomas: results of a pooled analysis from United States and European groups

Andrea Ferrari et al. Eur J Cancer. 2011 Mar.

Abstract

Background: Non-rhabdomyosarcoma soft tissue sarcomas (NRSTS) with initially unresected tumours represent a particular subset of patients with a poor outcome. Various international research groups pooled their data in a joint study in order to investigate prognostic variables and treatment modalities.

Methods: The study population consisted of 304 patients <21 years old treated between 1980 and 2005 using a multimodality therapeutic strategy.

Results: Synovial sarcoma and malignant peripheral nerve sheath tumour (MPNST) were the most frequent histotypes. Most patients received initial chemotherapy: major responses were recorded in 41% and minor in 16% of cases. Overall survival (OS) was 60.0% and 51.5% at 5 and 10 years, respectively, and it was significantly associated with patient's age, histological subtype, tumour site and size, quality of delayed surgical resection, radiotherapy administration and response to induction chemotherapy. MPNST associated to neurofibromatosis type 1 was the tumour type with the worst rate of response to chemotherapy and the worst outcome.

Conclusions: In unresected NRSTS patients, radiotherapy and delayed surgery are of crucial importance. Patients who respond to chemotherapy have better chance of survival. However, given the relatively poor prognosis, research on intensive multimodal treatment approaches and novel strategies is warranted.

PubMed Disclaimer

Figures

Fig. 1
Fig. 1
Kaplan-Meier overall survival curves according to histotype (panel A) and tumour site (panel B).
Fig. 2
Fig. 2
Kaplan-Meier overall survival curves according to quality of delayed surgery (panel A) and response to chemotherapy (panel B).
See this image and copyright information in PMC

References

    1. Okcu MF, Hicks J, Merchant TE, et al. Nonrhabdomyosarcomatous soft tissue sarcomas. In: Pizzo PA, Poplack DC, editors. Principles and practice of pediatric oncology. 5th Edn. Lippincott Williams and Wilkins; Philadelphia: 2006. pp. 1033–73.
    1. Ferrari A, Casanova M. New concepts for the treatment of pediatric non-rhabdomyosarcoma soft tissue sarcomas. Expert Rev Anticancer Ther. 2005;5:307–18. - PubMed
    1. Maurer HM, Beltangady M, Gehan EA, et al. The intergroup rhabdomyosarcoma study I: a final report. Cancer. 1988;61:209–20. - PubMed
    1. Ferrari A, Casanova M, Meazza C, et al. Adult-type soft tissue sarcomas in pediatric age: experience at the Istituto Nazionale Tumori in Milan. J Clin Oncol. 2005;23:4021–30. - PubMed
    1. Spunt SL, Ashley Hill D, Motosue AM, et al. Clinical features and outcome of initially unresected nonmetastatic pediatric nonrhaddomyosarcoma soft tissue sarcoma. J Clin Oncol. 2002;20:3225–35. - PubMed

Publication types

MeSH terms