Q131322881
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BACKGROUND: Validation of the prognostic value of the SIOPEN mIBG skeletal scoring system in two independent stage 4, mIBG avid, high-risk neuroblastoma populations. RESULTS: The semi-quantitative SIOPEN score evaluates skeletal meta-iodobenzylguanidine (mIBG) uptake on a 0-6 scale in 12 anatomical regions. Evaluable mIBG scans from 216 COG-A3973 and 341 SIOPEN/HR-NBL1 trial patients were reviewed pre- and post-induction chemotherapy. The prognostic value of skeletal scores for 5-year event free survival (5 yr.-EFS) was tested in the source and validation cohorts. At diagnosis, both cohorts showed a gradual non-linear increase in risk with cumulative scores. Several approaches were explored to test the relationship between score and EFS. Ultimately, a cutoff score of ≤3 was the most useful predictor across trials. A SIOPEN score ≤ 3 pre-induction was found in 15% SIOPEN patients and in 22% of COG patients and increased post-induction to 60% in SIOPEN patients and to 73% in COG patients. Baseline 5 yr.-EFS rates in the SIOPEN/HR-NBL1 cohort for scores ≤3 were 47% ± 7% versus 26% ± 3% for higher scores at diagnosis (p < 0.007) and 36% ± 4% versus 14% ± 4% (p < 0.001) for scores obtained post-induction. The COG-A3973 showed 5 yr.-EFS rates for scores ≤3 of 51% ± 7% versus 34% ± 4% for higher scores (p < 0.001) at diagnosis and 43% ± 5% versus 16% ± 6% (p = 0.004) for post-induction scores. Hazard ratios (HR) significantly favoured patients with scores ≤3 after adjustment for age and MYCN-amplification. Optimal outcomes were recorded in patients who achieved complete skeletal response. CONCLUSIONS: Validation in two independent cohorts confirms the prognostic value of the SIOPEN skeletal score. In particular, patients with an absolute SIOPEN score > 3 after induction have very poor outcomes and should be considered for alternative therapeutic strategies.
- MeSH
- 3-jodobenzylguanidin metabolismus MeSH
- biologický transport MeSH
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- neuroblastom diagnóza metabolismus MeSH
- předškolní dítě MeSH
- prognóza MeSH
- riziko MeSH
- společnosti lékařské * MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- lidé MeSH
- mladiství MeSH
- mužské pohlaví MeSH
- předškolní dítě MeSH
- ženské pohlaví MeSH
- Publikační typ
- časopisecké články MeSH
- klinické zkoušky MeSH
- validační studie MeSH
1st ed. 14, 393 s.
- MeSH
- diagnostické techniky radioisotopové * MeSH
- dítě MeSH
- kojenec MeSH
- Check Tag
- dítě MeSH
- kojenec MeSH
- Publikační typ
- monografie MeSH
- Konspekt
- Pediatrie
- NLK Obory
- pediatrie
- radiologie, nukleární medicína a zobrazovací metody
1st ed. xvi, 604 s.
- MeSH
- nukleární lékařství MeSH
- počítače MeSH
- Publikační typ
- monografie MeSH
- Konspekt
- Lékařské vědy. Lékařství
- NLK Obory
- radiologie, nukleární medicína a zobrazovací metody