Evaluation of the SIOPEN semi-quantitative scoring system in planar simpatico-adrenal MIBG scintigraphy in children with neuroblastoma.

Neuroblastoma is the most common malignancy in children comprising 7.6% of all infantile cancers. MIBG scintigraphy is a mandatory neuroblastoma diagnostic test, which is among others methods, semi-quantified by the SIOPEN method. The aim of this study was to test both the skeletal and the soft tissue segments of the SIOPEN scoring method in the diagnostic milieu and to correlate them with the Curie score. Since there is little knowledge of their diagnostic power, the following variables were tested: VMA, HVA, LDH, and MYCN, ferritin, bone marrow infiltration, the INSS and the INPC classification. The cross-sectional study with repeated measurements of 143 scintigrams was performed on 76 pediatric patients with suspected or proven neuroblastoma, who had been referred to the Center for Nuclear Medicine of the Clinical Center of Serbia in the period 2007-2012. The range of the SIOPEN soft tissue scores was 0-5. The range of the SIOPEN skeletal scores was 0-57. The range of the Curie scores was 0-26. The skeletal SIOPEN scores were significantly higher in bone marrow positive children, in children with pathologically elevated urinary VMA levels and in children having a more advanced clinical stage. There was no difference in the SIOPEN soft tissue score due to higher VMA levels, or depending on the clinical stage and positive bone marrow assessment. There was no difference between the SIOPEN skeletal and soft tissue scores on one hand and the histological grade of the tumor; elevated or normal levels of HVA, LDH, NSE and ferritin, or the presence or absence of MYNC amplification in the neuroblastoma cell line, on the other hand. The results of both SIOPEN scores showed a high linear correlation with the Curie score. The conclusion is that the soft tissue segment of the SIOPEN score needs further elucidation in a more controlled milieu. Excellent correlation between all segments of the two semi-quantitative scoring methods speaks in favor of the application of the complete SIOPEN scoring system in every day mIBG scanning.