Adrenal gland & paraganglia
General
Staging-neuroblastic tumors
Author: Debra L. Zynger, M.D.
Last author update: 1 January 2018
Last staff update: 2 February 2023
Copyright: 2002-2025, PathologyOutlines.com, Inc.
PubMed Search: Staging neuroblastic tumors
Table of Contents
International Neuroblastoma Staging System (INSS) | International Neuroblastoma Pathology Classification (INPC) histology group | Diagrams / tables | Additional referencesCite this page: Zynger D. Staging-neuroblastic tumors. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/adrenalstagingneuroblastic.html. Accessed April 2nd, 2025.
International Neuroblastoma Staging System (INSS)
- 1: localized tumor with complete gross (not necessarily microscopic) excision, negative representative ipsilateral lymph nodes microscopically; note: nodes removed with primary tumor may be positive; includes grossly resectable tumor in midline from pelvic ganglia or organ of Zuckerkandl
- 2A: localized tumor with incomplete gross excision, negative representative nonadherent ipsilateral lymph nodes microscopically; note: includes midline tumor that extends beyond one side of vertebral column and is unresectable
- 2B: localized tumor with positive representative nonadherent ipsilateral lymph nodes; enlarged contralateral lymph nodes must be negative microscopically; note: includes midline tumor that extends beyond one side of vertebral column, is unresectable with positive ipsilateral lymph node involvement (on side of extension); also includes a thoracic tumor with malignant unilateral pleural effusion
- 3: unresectable unilateral tumor infiltrating across midline or localized unilateral tumor with contralateral regional lymph node involvement; note: includes midline tumor with bilateral extension by infiltration (unresectable) or by lymph node involvement; includes a tumor of any size with malignant ascites or peritoneal implants
- 4: any primary tumor with dissemination to distant lymph nodes, bone, bone marrow, liver, skin or other organs (except 4S)
- 4S: localized primary tumor (stage 1, 2A or 2B) with dissemination limited to skin, liver or bone marrow (limited to infants < 1 year of age); note: marrow involvement should be minimal (< 10% of total nucleated cell identified as malignant); more extensive involvement should be classified as stage 4
- Note: for infants < 1 year, stages 1, 2A, 2B, 3 and 4S have similar rates of 4 year overall survival (98.5%) compared to stage 4 (73%)
International Neuroblastoma Pathology Classification (INPC) histology group
Favorable histopathology
Unfavorable histopathology
- Any age
- Ganglioneuroblastoma, intermixed
- Ganglioneuroma
- < 18 months
- Neuroblastoma, poorly differentiated subtype with low or intermediate mitosis-karyorrhexis index
- Neuroblastoma, differentiating subtype with low or intermediate mitosis-karyorrhexis index
- 18 - 60 months
- Neuroblastoma, differentiating subtype with low mitosis-karyorrhexis index
Unfavorable histopathology
- Any age
- Neuroblastoma, undifferentiated subtype
- Neuroblastoma, any subtype with high mitosis-karyorrhexis index
- 18 - 60 months
- Neuroblastoma, poorly differentiated subtype
- Neuroblastoma, differentiating subtype with intermediate mitosis-karyorrhexis index
- > 60 months
- Neuroblastoma, any subtype
Diagrams / tables
Images hosted on other servers:
Children's Oncology
Group (COG)
neuroblastoma risk
grouping system
INRG stage definitions
INRG consensus pretreatment classification
Additional references
- CAP: Cancer Protocol Templates [Accessed 26 January 2018], CAP: Protocol for the Examination of Specimens From Patients With Neuroblastoma [Accessed 26 January 2018], J Clin Oncol 2009;27:298, American Cancer Society: Neuroblastoma Risk Groups [Accessed 29 January 2018], INRG: International Neuroblastoma Risk Group Staging System [Accessed 29 January 2018], Pediatr Blood Cancer 2013;60:985, Oncologist 2003;8:278, J Clin Oncol 2009;27:289
