Pancreas
Neuroendocrine
TNM staging of neuroendocrine tumors of the pancreas

Author: Raul S. Gonzalez, M.D.
Deputy Editor Review: Debra Zynger, M.D.

Revised: 3 July 2018, last major update July 2018

Copyright: (c) 2018, PathologyOutlines.com, Inc.

PubMed Search: TNM staging of neuroendocrine tumors of the pancreas[title]

Cite this page: Gonzalez, R.S. TNM staging of neuroendocrine tumors of the pancreas. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pancreasneuroendocrinetumorstaging.html. Accessed November 20th, 2018.

Pathologic TNM staging of neuroendocrine tumors of the pancreas, AJCC 8th edition
Definition / general
  • All well differentiated neuroendocrine tumors of the pancreas are covered by this staging system
  • Not covered by this staging system are poorly differentiated neuroendocrine carcinomas at this location (use the pancreas exocrine staging system instead)
Essential features
  • AJCC 7th edition staging was sunset on December 31, 2017; as of January 1, 2018, use of the AJCC, 8th Edition, 2018 is mandatory
ICD-10 coding
  • C25.4: malignant neoplasm of endocrine pancreas
Primary tumor (pT)
  • TX: tumor cannot be assessed
  • T1: tumor limited to the pancreas, < 2 cm
  • T2: tumor limited to the pancreas, 2 - 4 cm
  • T3: tumor limited to the pancreas, > 4 cm; or tumor invading the duodenum or common bile duct
  • T4: tumor invading adjacent organs (stomach, spleen, colon, adrenal gland) or the wall of large vessels (celiac axis or the superior mesenteric artery)

Note:
  • "Limited to the pancreas" means no extension into adjacent organs or structures; invasion of peripancreatic adipose tissue does not impact staging
Regional lymph nodes (pN)
  • NX: regional lymph nodes cannot be assessed
  • N0: no regional lymph node involvement
  • N1: regional lymph node involvement

Notes:
  • Regional lymph nodes depend on tumor site
    • For tumors of the head of the pancreas, regional nodes include common bile duct, common hepatic artery, portal vein, posterior and anterior pancreatoduodenal arcades, superior mesenteric vein and right lateral wall of superior mesenteric vein nodes
    • For tumors of the body or tail of the pancreas, regional nodes include common hepatic artery, celiac axis, splenic artery and splenic hilum nodes
Distant metastasis (pM)
  • M0: no distant metastasis
  • M1: distant metastasis
    • M1a: metastases confined to the liver
    • M1b: metastases in at least one extrahepatic site (e.g., lung, ovary, nonregional lymph node, peritoneum, bone)
    • M1c: both hepatic and extrahepatic metastases
Prefixes
  • r: recurrent tumor stage
Stage grouping
  • Stage I:T1 N0 M0
  • Stage II:T2 - 3 N0 M0
  • Stage III:T4 N0 M0
  • Any T N1 M0
  • Stage IV:Any T Any N M1

Registry data collection variables
  • Size of tumor (value, unknown)
  • Presence of invasion into adjacent organs / structures (stomach, duodenum, spleen, colon, other) and whether multiple adjacent organs were involved
  • Presence of necrosis
  • Number of tumors (multicentric disease at primary site)
  • Lymph node status (including number of lymph nodes assessed and number of positive nodes)
  • Grade (based on Ki67 or mitotic count; G1, G2, G3, unknown)
  • Mitotic count (value; unknown)
  • Ki67 labeling index (value; unknown)
  • Perineural invasion
  • Lymphovascular invasion
  • Margin status
  • Functional status (type of syndrome)
  • Genetic syndrome (type of syndrome)
  • Location in pancreas (head, tail, body, junction body / tail, junction body / head, unknown)
  • Type of surgery (enucleation, distal pancreatectomy, with or without splenectomy, central pancreatectomy, pancreaticoduodenectomy / Whipple procedure, other)
  • Preoperative CgA level (absolute value with upper limit of normal; unknown)
  • Age of patient
Histologic grade
  • Grading is not formally part of the staging system
  • Most pathologists use the ENETS / WHO grading criteria:
    • Grade 1: mitotic rate < 2 per 10 high power fields and Ki67 rate < 3%
    • Grade 2: mitotic rate 2 - 20 per 10 high power fields or Ki67 rate 3 - 20%
    • Grade 3: mitotic rate > 20 per 10 high power fields or Ki67 rate > 20%
Board review question #1
Invasion of which of the following structures would upstage a pancreatic neuroendocrine tumor to pT4 disease?

  1. Common bile duct
  2. Duodenum
  3. Peripancreatic fat
  4. Spleen
Board review answer #1
D. Spleen