The European study, LINES 2009 (Low and Intermediate Risk Neuroblastoma European Study),
groups together in a single protocol the treatment of all patients with "non high risk"
neuroblastoma (NB), with stratification into two groups: low risk and intermediate risk.
These two separate cohorts are included in one single protocol to enable patient data from
these two groups to be entered into a common database, as the current prognostic
classifications determining treatment may evolve further with subsequent more detailed
molecular analysis of the tumours.
1. LOW RISK STUDY
The Low Risk Study is proposed in order to:
- minimise the amount of treatment (chemotherapy and surgery) for all appropriate low risk
patients, who in previous studies have been shown to have an excellent long-term outcome
(as in the SIOPEN 99.1-2 infant neuroblastoma studies where the overall survival was
greater than 97%(H. Rubie, JCO).
- improve the EFS and maintain the OS (overall survival) in L2 and Ms patients with a
SCA(Segmental Cromosomal Aberration) genomic profile tumour (presence of any segmental
chromosomal change (SCA)) by electively treating these patients with chemotherapy
despite the absence of symptoms.
2) INTERMEDIATE RISK STUDY
The Intermediate Risk Study is proposed in order to:
- reduce the amount of chemotherapy for differentiating histology INRG (International
Neuroblastoma Risk Group) stage L2 NB and ganglioneuroblastoma nodular patients who in
previous SIOPEN study have been shown to have an excellent long-term outcome;
- increase the amount of treatment (radiotherapy and 13-cis-RA (13-cis-Retinoic Acid) for
poorly differentiated or undifferentiated histology INRG stage L2 NB or
ganglioneuroblastoma nodular patients in order to improve the EFS registered in the
previous SIOPEN study;
- improve the EFS (Event Free Survival) of MYCN (V-Myc myelocytomatosis viral related
oncogene, NB derived ,avian )amplified INSS (International NB Staging System) stage 1 NB
patients with the introduction of adjuvant treatment;
- maintain the very good results obtained in previous SIOPEN study for INRG stage M
infants with a moderate treatment.
NEONATAL SUPRARENAL MASSES
The incidence of suprarenal tumours/masses has increased in the last decade due to the
expanded use of prenatal ultrasonography in routine obstetric care and in the neonatal and
early infancy care. The differential diagnosis of these masses ranges from benign (adrenal
haemorrhage) to malignant processes (neuroblastoma, adrenal carcinoma). Knowledge on
perinatal suprarenal masses, although based on a relatively large literature, is scattered
amongst studies on very few cases with no methodical approach and often short follow up.
Therefore, the optimal management of these masses has not been clearly defined. Neuroblastoma
at this age is an intriguing entity with a very good prognosis in most cases. The SIOPEN
Group, based on their results in the first multicenter European Trial for infants with
neuroblastoma (INES) and the world-wide experience provided in the literature, is launching
this European surveillance study (Multi-centre, non-blinded, one armed prospective trial) for
these masses. Treatment: Observation