TY - JOUR
T1 - Characteristics and outcome of stage II and III non-anaplastic Wilms' tumour treated according to the SIOP trial and study 93-01
AU - Graf, Norbert
AU - Van Tinteren, Harm
AU - Bergeron, Christophe
AU - Pein, François
AU - Van Den Heuvel-Eibrink, Marry M.
AU - Sandstedt, Bengt
AU - Schenk, Jens Peter
AU - Godzinski, Jan
AU - Oldenburger, Foppe
AU - Furtwängler, Rhoikos
AU - De Kraker, Jan
N1 - Funding Information:
Role of the funding sources: The study was funded in part by the ‘ Deutsche Krebshilfe ’ project number: 70-1899 for the conduct in GPOH. The Nederlandse Stichting Kindergeneeskundig Kankeronderzoek number 86-04 for the conduct in the other countries and statistical analysis. Barncancerfonden, Sweden for supporting reference pathology.
Funding Information:
The concept of neoadjuvant chemotherapy has an important role in the treatment of most paediatric solid tumours. Complete removal of a shrunken tumour is facilitated, risk of mutilation and ruptures caused by surgical procedures is minimised or avoided, and micrometastases, not visible at diagnosis, are treated as early as possible. As shown above the in vivo response to preoperative chemotherapy is reflected in the histological subtyping and the tumour volume. Both parameters serve as early individual prognostic markers and make it possible to stratify and individualise postoperative treatment. In the near future biological markers will probably be added to predict response to treatment upfront and to stratify patients according to such risk factors as primarily shown by NWTSG/COG. 14,29 New molecular and information technologies will facilitate the integration of all available data of a patient and enhance the development of models to simulate and predict the response of treatment in individual patients. This will pave the way to personalised treatment 30,31 as investigated in the current project p-medicine funded by the European Commission under Framework Programme 7. 32
PY - 2012/11
Y1 - 2012/11
N2 - Purpose: To determine the prognosis of children with stage II and III of low or intermediate risk histology (SIOP classification) in unilateral localised Wilms tumour (WT) after neoadjuvant chemotherapy according to the trial and study of the International Society of Paediatric Oncology, SIOP 93-01. Patients and methods: Patients with unilateral localised WT and stage II or III with low (LR) or intermediate risk (IR) histology between 6 months and 18 years of age, were selected from the total sample of patients registered in the SIOP 93-01 study between June 1993 and December 2001. All patients received 4 weeks of actinomycin-D/vincristine before surgery. Postoperative chemotherapy consisted of actinomycin-D, vincristine and epirubicin/doxorubicin for 27 weeks. Flank or whole abdomen irradiation was given for stage III. Event-free survival (EFS) and overall survival (OS) were analysed for various subgroups. Results: Of 1476 registered patients 594 (40%) met the inclusion criteria for this analysis. Four hundred and two (67%) had stage II disease and 563 (95%) had intermediate risk histology. Median tumour volume was 439 ml at diagnosis and 163 ml after preoperative chemotherapy. With a median follow-up of 8 years, 5-year EFS was 90% (95% confidence interval [95% CI]: 87-92%) and OS 95% (95% CI: 93-97%). Patients with stage III, blastemal type histology and a large volume at surgery had a worse outcome. Conclusion: Treatment for stage II and III LR or IR WT is successful in a neoadjuvant setting as advised by the SIOP. Stage, tumour volume and blastemal type histology are the most important prognostic factors.
AB - Purpose: To determine the prognosis of children with stage II and III of low or intermediate risk histology (SIOP classification) in unilateral localised Wilms tumour (WT) after neoadjuvant chemotherapy according to the trial and study of the International Society of Paediatric Oncology, SIOP 93-01. Patients and methods: Patients with unilateral localised WT and stage II or III with low (LR) or intermediate risk (IR) histology between 6 months and 18 years of age, were selected from the total sample of patients registered in the SIOP 93-01 study between June 1993 and December 2001. All patients received 4 weeks of actinomycin-D/vincristine before surgery. Postoperative chemotherapy consisted of actinomycin-D, vincristine and epirubicin/doxorubicin for 27 weeks. Flank or whole abdomen irradiation was given for stage III. Event-free survival (EFS) and overall survival (OS) were analysed for various subgroups. Results: Of 1476 registered patients 594 (40%) met the inclusion criteria for this analysis. Four hundred and two (67%) had stage II disease and 563 (95%) had intermediate risk histology. Median tumour volume was 439 ml at diagnosis and 163 ml after preoperative chemotherapy. With a median follow-up of 8 years, 5-year EFS was 90% (95% confidence interval [95% CI]: 87-92%) and OS 95% (95% CI: 93-97%). Patients with stage III, blastemal type histology and a large volume at surgery had a worse outcome. Conclusion: Treatment for stage II and III LR or IR WT is successful in a neoadjuvant setting as advised by the SIOP. Stage, tumour volume and blastemal type histology are the most important prognostic factors.
KW - Local stage II and III
KW - Neoadjuvant treatment
KW - Prognosis
KW - Wilms tumour
UR - http://www.scopus.com/inward/record.url?scp=84868210723&partnerID=8YFLogxK
U2 - 10.1016/j.ejca.2012.06.007
DO - 10.1016/j.ejca.2012.06.007
M3 - Article
C2 - 22795263
AN - SCOPUS:84868210723
SN - 0959-8049
VL - 48
SP - 3240
EP - 3248
JO - European Journal of Cancer
JF - European Journal of Cancer
IS - 17
ER -