HOMEPRODUCTSSERVICESCOMPANYCONTACTFAQResearchDictionaryPharmaMobileSign Up FREE or Login

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

AbstractBACKGROUND:
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.
AuthorsAndrea Ferrari, 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
JournalEuropean journal of cancer (Oxford, England : 1990) (Eur J Cancer) Vol. 47 Issue 5 Pg. 724-31 (Mar 2011) ISSN: 1879-0852 [Electronic] England
PMID21145727 (Publication Type: Journal Article, Meta-Analysis, Multicenter Study)
CopyrightCopyright © 2010 Elsevier Ltd. All rights reserved.
Topics
  • Adolescent
  • Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
  • Chemotherapy, Adjuvant
  • Child
  • Child, Preschool
  • Combined Modality Therapy
  • Female
  • Humans
  • Infant
  • Kaplan-Meier Estimate
  • Male
  • Neoplasm Recurrence, Local (etiology)
  • Nerve Sheath Neoplasms (pathology, therapy)
  • Sarcoma (pathology, therapy)
  • Sarcoma, Synovial (therapy)
  • Treatment Outcome
  • United States
  • Young Adult

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research network!


Choose Username:
Email:
Password:
Verify Password: