Clinical value of INSS/INRGSS staging of peripheral neuroblastictumors in children

Abstract

Objective To explore the role of International Neuroblastoma Staging System (INSS) or International Neuroblastoma Risk Group Staging System (INGRSS) in peripheral neuroblastic tumors. Methods From 2006 to 2018, a retrospective analysis was performed for 108 peripheral neuroblastictumors (pNT) children.There were 62 boys and 46 girls.All of them were operated and pathological examinations conducted.However, 4 s/Ms staged cases were excluded.Analyses were performed with regards to age, weight distribution, pathological results, INSS/INRGSS stage, neuron-specific enolase (NSE), lactate dehydrogenase (LDH), lesion size, image-defined risk factors (IDRFs), operative duration and tumor index.The relationship between stage and complete removal was explored. Results No statistically significant inter-gender difference existed in the distribution between tumors and the age of neuroblastoma (NB) was younger.The specific pathologies were NB (n=68), ganglioneuroblastoma (GNB, n=30) and ganglioneuroma (GN, n=10). No significant difference existed in the distribution of INSS/INRGSS staging.NSE and LDH values of pNTs with diameters >5 cm and L2 & M phases spiked sharply.The number of IDRFs was higher in NB than GNB.And pNT patients with diameters >10 cm, stages L2 & M and positive IDRFs had a longer operative duration and a lower probability of complete resection. Conclusions The preoperative evaluations of INSS/INRGSS staging for pNT offer more advantages than INSS staging alone.It may guide the choice of specific treatments.The presence or absence of IDRFs is an important factor for a complete removal of tumors. Key words: Neuroblastoma; Peripheral neuroblastictumors; Ganglioneuroblastoma; Ganglioneuroma