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Cite this page: Gonzalez RS. Staging-neuroendocrine. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasneuroendocrinetumorstaging.html. Accessed March 22nd, 2023.
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 coding
- ICD-10: C25.4 - malignant neoplasm of endocrine pancreas
Primary tumor (pT)
- pTX: tumor cannot be assessed
- pT1: tumor limited to the pancreas, < 2 cm
- pT2: tumor limited to the pancreas, 2 - 4 cm
- pT3: tumor limited to the pancreas, > 4 cm; or tumor invading the duodenum or common bile duct
- pT4: 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)
- pNX: regional lymph nodes cannot be assessed
- pN0: no regional lymph node involvement
- pN1: 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
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 style question #1
Invasion of which of the following structures would upstage a pancreatic neuroendocrine tumor to pT4 disease?
- Common bile duct
- Duodenum
- Peripancreatic fat
- Spleen
Board review style answer #1
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