International Neuroblastoma Risk Group Staging System (INRGSS)
This input is used in derivation
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)NAACCR Item
NAACCR #9611Metadata
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 |