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.
Spleen shows a high physiological uptake on radionuclide somatostatin receptor (SSTR) imaging studies. Autoradiography and immunohistochemistry studies showed that SSTRs are mainly located in the red pulp of the spleen. In this review article we will summarize the significance of splenic uptake in SSTR imaging studies and will also present high resolution splenic images of Ga-68 DOTANOC PET in which splenic distribution of the radiotracer appears to be correlating with the distribution of red pulp.
3D echocardiography is applicable in the assessment of both preserved and reduced left ventricular ejection fraction. This assessment is fast and requires minimal user intervention. 3D strain may help in cardiac resynchronization therapy candidates and response assessment. After cardiac resynchronization, none of the patients were incorrectly identified as responder to cardiac resynchronization therapy by 3D algorithms compared to 2D Simpson's reference.
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