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Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study.
Eur J Cancer. 2006 May; 42(8):1120-8.EJ

Abstract

The SIOP PNET 3 study was designed to determine whether 10 weeks of moderately intensive chemotherapy given after surgery and before radiotherapy (RT) would improve the outcome for patients with primitive neuroectodermal tumours (PNETs) compared with RT alone. Patients with a histological diagnosis of supratentorial PNET (StPNET) and no radiological evidence of metastatic disease were initially eligible for randomisation to either chemotherapy followed by craniospinal RT 35 Gy in 21 fractions with a boost of 20 Gy in 12 fractions to the primary site, or RT alone. In respect of the increasing recognition that StPNET were high-risk tumours, randomisation for this group closed in November 1999. This analysis includes both randomised and non-randomised patients with StPNET entered into the study database. Sixty-eight patients aged 2.9-16.6 years (median 6.5 years) were included in the analysis (chemotherapy+RT: 44, RT alone: 24). Fifty-four patients (79%) had a non-pineal and 14 (21%) a pineal site. At a median follow-up of 7.4 years, for all patients overall survival (OS) at 3 and 5 years was 54.4% and 48.3%, respectively. Event-free survival (EFS) at 3 and 5 years was 50.0% and 47.0%, respectively. There was no statistically significant difference in OS or EFS according to treatment received. OS (P=0.05) and EFS (P=0.03) were significantly better for patients with pineal primary sites. EFS for pineal tumours were 92.9% at 3 years and 71.4% at 5 years and for non-pineal primaries 40.7% at 3 years and 40.7% at 5 years. This study confirmed the relatively good survival for non-metastatic pineal PNETs but poor survival of non-pineal StPNETs. There was no evidence that pre-radiation chemotherapy improved outlook. Future treatment programs should be directed at the particular natural history of these tumours, to further define prognostic factors and to explore further biological characteristics.

Authors+Show Affiliations

Alder Hey's Children's Hospital-Oncology Unit, Eaton Road, Liverpool L12 2AP, UK. bpizer@liv.ac.ukNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article
Randomized Controlled Trial
Research Support, Non-U.S. Gov't

Language

eng

PubMed ID

16632346

Citation

Pizer, Barry L., et al. "Analysis of Patients With Supratentorial Primitive Neuro-ectodermal Tumours Entered Into the SIOP/UKCCSG PNET 3 Study." European Journal of Cancer (Oxford, England : 1990), vol. 42, no. 8, 2006, pp. 1120-8.
Pizer BL, Weston CL, Robinson KJ, et al. Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study. Eur J Cancer. 2006;42(8):1120-8.
Pizer, B. L., Weston, C. L., Robinson, K. J., Ellison, D. W., Ironside, J., Saran, F., Lashford, L. S., Tait, D., Lucraft, H., Walker, D. A., Bailey, C. C., & Taylor, R. E. (2006). Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study. European Journal of Cancer (Oxford, England : 1990), 42(8), 1120-8.
Pizer BL, et al. Analysis of Patients With Supratentorial Primitive Neuro-ectodermal Tumours Entered Into the SIOP/UKCCSG PNET 3 Study. Eur J Cancer. 2006;42(8):1120-8. PubMed PMID: 16632346.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study. AU - Pizer,Barry L, AU - Weston,Claire L, AU - Robinson,Kathryn J, AU - Ellison,David W, AU - Ironside,James, AU - Saran,Frank, AU - Lashford,Linda S, AU - Tait,Diana, AU - Lucraft,Helen, AU - Walker,David A, AU - Bailey,Clifford C, AU - Taylor,Roger E, Y1 - 2006/04/24/ PY - 2005/09/21/received PY - 2005/11/29/revised PY - 2006/01/03/accepted PY - 2006/4/25/pubmed PY - 2006/7/29/medline PY - 2006/4/25/entrez SP - 1120 EP - 8 JF - European journal of cancer (Oxford, England : 1990) JO - Eur J Cancer VL - 42 IS - 8 N2 - The SIOP PNET 3 study was designed to determine whether 10 weeks of moderately intensive chemotherapy given after surgery and before radiotherapy (RT) would improve the outcome for patients with primitive neuroectodermal tumours (PNETs) compared with RT alone. Patients with a histological diagnosis of supratentorial PNET (StPNET) and no radiological evidence of metastatic disease were initially eligible for randomisation to either chemotherapy followed by craniospinal RT 35 Gy in 21 fractions with a boost of 20 Gy in 12 fractions to the primary site, or RT alone. In respect of the increasing recognition that StPNET were high-risk tumours, randomisation for this group closed in November 1999. This analysis includes both randomised and non-randomised patients with StPNET entered into the study database. Sixty-eight patients aged 2.9-16.6 years (median 6.5 years) were included in the analysis (chemotherapy+RT: 44, RT alone: 24). Fifty-four patients (79%) had a non-pineal and 14 (21%) a pineal site. At a median follow-up of 7.4 years, for all patients overall survival (OS) at 3 and 5 years was 54.4% and 48.3%, respectively. Event-free survival (EFS) at 3 and 5 years was 50.0% and 47.0%, respectively. There was no statistically significant difference in OS or EFS according to treatment received. OS (P=0.05) and EFS (P=0.03) were significantly better for patients with pineal primary sites. EFS for pineal tumours were 92.9% at 3 years and 71.4% at 5 years and for non-pineal primaries 40.7% at 3 years and 40.7% at 5 years. This study confirmed the relatively good survival for non-metastatic pineal PNETs but poor survival of non-pineal StPNETs. There was no evidence that pre-radiation chemotherapy improved outlook. Future treatment programs should be directed at the particular natural history of these tumours, to further define prognostic factors and to explore further biological characteristics. SN - 0959-8049 UR - https://www.unboundmedicine.com/medline/citation/16632346/Analysis_of_patients_with_supratentorial_primitive_neuro_ectodermal_tumours_entered_into_the_SIOP/UKCCSG_PNET_3_study_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0959-8049(06)00208-5 DB - PRIME DP - Unbound Medicine ER -