Adrenal Ganglioneuroblastoma in Adults: A Case Report and Review of the Literature

Table 2

International Neuroblastoma Risk Group Consensus Pretreatment Classification Scheme.

INRG Stage	Age (months)	INPC group	Grade of differentiation	MYCN gene	11q aberration	Ploidy	Pretreatment risk group
L1/L2		GN maturing; GNB intermixed					Very low
L1; Localized tumor confined to one body compartment and with absence of image-defined risk factors		Any, except GN maturing or GNB intermixed		NA; Amplified			Very low; High
L2; Locoregional tumor with presence of one or more image-defined risk factor	<18	Any, except GN maturing or GNB intermixed		NA	No		Low
					Yes		Intermediate
	≥18	GNB nodular; NB	Differentiating	NA	No		Low
					Yes		Intermediate
			Poorly differentiated or undifferentiated	NA
				Amplified			High
M; Distant metastatic disease (except MS)	<18			NA		Hyperploid	Low
	<12			NA		Diploid	Intermediate
	12–18			NA		Diploid	Intermediate
	<18			Amplified			High
	≥18						High
MS; Metastatic disease confined to skin, liver, and/or bone marrow in children <18 months				NA	no		Very low
					yes		High
				Amplified			High

INRG: International Neuroblastoma Risk Group; INCP: International Neuroblastoma Pathology Classification; GN = ganglioneuroma; GNB = ganglioneuroblastoma; NB = neuroblastoma; NA: not amplified; modified from Cohn et al. [11].