International Neuroblastoma Risk Group Staging System

Stage	Description
L1	Localized tumor not involving vital structures as defined by the list of IDRFs (below) and confined to one body compartment
L2	Locoregional tumor with presence of one or more IDRFs
M	Distant metastatic disease (except stage MS)
MS	Metastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow
IDRFs in neuroblastic tumors
Ipsilateral tumor extension within two body compartments
Neck-chest, chest-abdomen, abdomen-pelvis
Neck
Tumor encasing carotid and/or vertebral artery and/or internal jugular vein
Tumor extending to base of skull
Tumor compressing the trachea
Cervicothoracic junction
Tumor encasing brachial plexus roots
Tumor encasing subclavian vessels and/or vertebral and/or carotid artery
Tumor compressing the trachea
Thorax
Tumor encasing the aorta and/or major branches
Tumor compressing the trachea and/or principal bronchi
Lower mediastinal tumor, infiltrating the costovertebral junction between T9 and T12
Thoraco-abdominal
Tumor encasing the aorta and/or vena cava
Abdomen/pelvis
Tumor infiltrating the porta hepatis and/or the hepatoduodenal ligament
Tumor encasing branches of the superior mesenteric artery at the mesenteric root
Tumor encasing the origin of the coeliac axis, and/or of the superior mesenteric artery
Tumor invading one or both renal pedicles
Tumor encasing the aorta and/or vena cava
Tumor encasing the iliac vessels
Pelvic tumor crossing the sciatic notch
Intraspinal tumor extension whatever the location provided that:
More than one third of the spinal canal in the axial plane is invaded and/or the perimedullary leptomeningeal spaces are not visible and/or the spinal cord signal is abnormal
Infiltration of adjacent organs/structures
Pericardium, diaphragm, kidney, liver, duodenopancreatic block, and mesentery
Conditions to be recorded, but not considered IDRFs
Multifocal primary tumors
Pleural effusion, with or without malignant cells
Ascites, with or without malignant cells

Patients with multifocal primary tumors should be staged according to the greatest extent of disease as defined in the table.

IDRFs: image-defined risk factors.

Reproduced with permission from: Monclair T, Brodeur GM, Ambros PF, et al. The International Neuroblastoma Risk Group (INRG) Staging System: An INRG Task Force Report. J Clin Oncol 2009; 27:298. Copyright © 2009 American Society of Clinical Oncology.

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International Neuroblastoma Risk Group Staging System