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Version 25
Data Item #1185: Intl Neuroblastoma Risk Grp Stag Sys (INRGSS)

* indicates required field

Length: 1
Source of Standard: NAACCR/SEER
Section Name: Stage/Prognostic Factors
Year Implemented: 2025
Version Implemented: 25
Record Types: C, A, I, M

XML NAACCR ID: inrgss

Parent XML Element: Tumor

Alternate Names:

  • INRG

Required Status:

NPCR Collect: . - No recommendations
CoC Collect: . - No recommendations
SEER Collect: RS* - Required, site specific; when available
CCCR Collect: . - No recommendations

Description:

International Neuroblastoma Risk Group Staging System (INRGSS) for Neuroblastoma is defined based on clinical work up and image-defined risk factors. 

The INRGSS is a clinically 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

Rationale:

This is part of the National Childhood Cancer Registry (NCCR) project to collect more specific information on pediatric patients. Registries part of the NCCR will start collection on specific pediatric data items with 2024+ diagnoses.

Item Data Type:

numeric

Allowable Values

1, 2, 3, 4, 8, 9

Codes:

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)
  • 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

Code Notes:

Note 1: Effective years

  • This SSDI is effective for diagnosis years 2024+
  • For cases diagnosed 2018-2023, this SSDI must be blank

Note 2: Image-Defined Risk Factors

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 pleural effusion

  • 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: Lymph node involvement

  • Regional lymph node involvement does not factor into staging
  • Code 3 for non-regional lymph node involvement (distant lymph nodes)