Cite this page: Gonzalez RS. Staging-neuroendocrine. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/pancreasneuroendocrinetumorstaging.html. Accessed December 26th, 2024.
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 8th edition is mandatory
- AJCC 9th edition was released in 2023, with minimal changes from the 8th edition
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 in greatest dimension
- pT2: tumor limited to the pancreas, > 2 cm but ≤ 4 cm in greatest dimension
- pT3: tumor limited to the pancreas, > 4 cm in greatest dimension or tumor invading the duodenum, ampulla of Vater or common bile duct
- pT4: tumor invading adjacent organs (stomach, spleen, colon, adrenal gland) or the wall of large vessels (celiac axis, superior mesenteric artery / vein, splenic artery / vein, gastroduodenal artery / vein, portal vein)
Notes:
- Limited to the pancreas means no extension into adjacent organs or structures; invasion of peripancreatic adipose tissue does not impact staging
- When multiple tumors are present, the largest tumor should be used to assign the T category
- Use T(#) (e.g., pT3[4] N0 M0) or use the m suffix, T(m) (e.g., pT3[m] N0 M0)
Regional lymph nodes (pN)
- NX: regional lymph nodes cannot be assessed
- N0: no tumor involvement of regional lymph node(s)
- N1: tumor involvement of regional lymph node(s)
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
Prefixes
- c: clinical
- p: pathological
- yc: posttherapy clinical
- yp: posttherapy pathological
Primary tumor suffix
- (m): multiple synchronous primary tumors
Regional lymph nodes suffix
- (f): fine needle aspiration (FNA) or core needle biopsy
AJCC prognostic stage groups
| Stage I: | T1 | N0 | M0
|
| Stage II: | T2, T3 | N0 | M0
|
| Stage III: | T4 | N0 | M0
|
| | any T | N1 | M0
|
| Stage IV: | any T | any N | M1
|
Prognostic tumor characteristics
- Mitotic count
- Ki67 index
- Associated genetic syndrome
- Chromogranin A (CgA)
- Functionality
- DAXX / ATRX
- ARX, PDX1 expression
Registry data collection variables
- Size of tumor (value, unknown)
- Presence of invasion into adjacent organs / structures
- If yes, which ones (pick all that apply)
- Stomach (yes / no)
- Duodenum (yes / no)
- Spleen (yes / no)
- Colon (yes / no)
- Other:
- If yes, were multiple adjacent organs involved (yes / no)
- 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 index (value; unknown)
- Perineural invasion
- Lymphovascular invasion
- Margin status
- Functional status (yes / no, type of syndrome)
- Genetic syndrome (yes / no, 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, unknown, other)
- Preoperative CgA level (absolute value with upper limit of normal; unknown)
- Age of patient
Histologic grade (G)
- G1: mitotic rate < 2 per 2 mm2 and Ki67 < 3%
- G2: mitotic rate 2 - 20 per 2 mm2 or Ki67 3 - 20%
- G3: mitotic rate > 20 per 2 mm2 or Ki67 > 20%
Histopathologic type
- Neuroendocrine tumor (NET), NOS
- Neuroendocrine tumor, grade 1
- Neuroendocrine tumor, grade 2
- Neuroendocrine tumor, grade 3
- Pancreatic neuroendocrine tumor, nonfunctioning
- Insulinoma
- Gastrinoma
- Vasoactive intestinal peptide secreting tumor (VIPoma)
- Glucagonoma
- Somatostatinoma
- Adrenocorticotropic hormone (ACTH) producing neuroendocrine tumor
- Serotonin producing neuroendocrine tumor
- Growth hormone (GH) producing neuroendocrine tumor
Residual tumor (operative factor)
- R0: complete resection, margins histologically negative, no residual tumor left after resection
- R1: incomplete resection, margins histologically involved, microscopic tumor remains after resection of gross disease (relevant to resection margins that are microscopically involved by tumor)
- R2: incomplete resection, margins involved or gross disease remains
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
D. Spleen. Direction invasion of this organ is specifically noted as upstaging a pancreatic well differentiated neuroendocrine tumor to pT4 disease. Answers A and B are incorrect because invasion of these structures is noted as signifying pT3 disease. Answer C is incorrect because invasion of peripancreatic fat does not affect the staging of these tumors.
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Reference:
Pancreas - Staging-neuroendocrine Board review style question #2
Which of the following is true regarding staging and prognosis of pancreatic well differentiated neuroendocrine tumors?
- ARX and PDX1 expression are considered prognostic indicators
- More than 5 positive lymph nodes indicates pN2 disease
- Regional lymph nodes are the same regardless of tumor location within the pancreas
- Tumor functionality is not considered a prognostic indicator
Board review style answer #2
A. ARX and PDX1 expression are considered prognostic indicators per the AJCC, among multiple additional factors. Answer B is incorrect because there is no pN2 designation for pancreatic neuroendocrine tumors. Answer C is incorrect because the regional lymph nodes are different depending on whether the tumor arises in the head or the body / tail. Answer D is incorrect because tumor functionality is considered a prognostic indicator.
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Reference:
Pancreas - Staging-neuroendocrine
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