International Neuroblastoma Risk Group Staging System (INRGSS)
Notes
**Note 1:** This SSDI is effective for diagnosis years 2024+
* For cases diagnosed 2018-2023, leave this SSDI blank
**Note 2:** The INRGSS is a clinical based staging system and is able to be utilized for every single neuroblastoma tumor as it is based on the clinical work up and image-defined risk factors
* Primary source of information for this data item is imaging
* Do not code any staging information from results of surgical resection in this data item
- **Note:** There is a different staging system available for patients who have surgery (International Neuroblastoma Staging System (INSS)) which is collected in Pediatric Primary Tumor, Pediatric Regional Nodes, and Pediatric Mets
**Note 3:** Image-Defined Risk Factors in Neuroblastic Tumors: Staging requires assessment of whether patients have none, or one or more of the image-defined risk factors (IDRF). These IDRF’s are based on imaging prior to any surgical resection or other treatment.
* 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
* Cervico-thoracic 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 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, duodeno-pancreatic block, and mesentery
**Note 4:** Ascites and/or a pleural effusion, even with malignant cells, do not constitute metastatic disease unless they are remote from the body compartment of the primary tumor.
**Note 5:** Regional lymph node involvement does not factor into staging
* Code 3 for non-regional lymph node involvement (distant lymph nodes)
Metadata
SSDI
| Code |
Description |
| 1 |
Stage L1
* Localized tumor that does not involve any vital structures
* Tumor confined within one body compartment (i.e., neck, chest, abdomen, or pelvis)
* No evidence of image-defined risk factors (IDRF’s)
* Intraspinal tumor extension that does not fulfil the criteria for an IDRF is consistent with stage (L1) |
| 2 |
Stage L2
* Locoregional tumor with evidence of image-defined risk factors (IDRF’s)
* Tumor ipsilaterally contiguous within body compartments (i.e., a left sided abdominal tumor with left-sided lung, bone or pleura involvement)
- Non-contiguous disease would be coded as M (e.g. left sided abdominal tumor with right-sided lung, bone or pleura) |
| 3 |
Stage M
* Distant metastatic disease (see Stage MS for patients less than 18 months old)
* Noncontiguous disease
* Distant lymph node involvement |
| 4 |
Stage MS
* For patients less than 18 months only (547 days) metastatic disease confined to
- Bone marrow
+ MIBG scintigraphy must be negative in bone and bone marrow
- Skin
- Liver |
| 8 |
Not applicable: Information not collected for this case
(If this information is required by your standard setter, use of code 8 may result in an edit error) |
| 9 |
Not documented in medical record
International Neuroblastoma Risk Group Staging System not assessed
Death certificate only |
| <BLANK> |
N/A - Diagnosis year is prior to 2024 |
(1) American Society of Clinical Oncology © 2009, The International Neuroblastoma Risk Group (INRG) Classification System: AN INRG Task Force Report; published by American Society of Clinical Oncology, 2009
(2) Aitken JF, Youlden D, O’Neill L, Gupta S, Frazier AL, eds. Childhood cancer staging for population registries according to the Toronto Childhood Cancer Stage Guidelines – Version 2. Cancer Council Queensland and Cancer Australia: Brisbane, Australia; 2021.
