Small intestine & ampulla
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Staging-duodenal & ampullary neuroendocrine tumors
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PubMed Search: TNM staging ampullary neuroendocrine tumors
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Cite this page: Gonzalez RS. Staging-duodenal & ampullary neuroendocrine tumors. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/ampullatnmstaging.html. Accessed May 30th, 2023.
Pathologic TNM staging of neuroendocrine tumors of the duodenum and ampulla of Vater, AJCC 8th edition
Definition / general
- Well differentiated neuroendocrine tumors of the ampulla ("carcinoids") and of the duodenum are covered by this staging system
- Not covered by this staging system are poorly differentiated neuroendocrine carcinomas at this location (use the adenocarcinoma 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
- Hormone secretion by the tumor (gastrin, somatotatin, etc.) is not incorporated into staging
Terminology
- "Carcinoid” is no longer an acceptable term
ICD coding
- ICD-10: C7A.019 - Malignant carcinoid tumor of the small intestine, unspecified portion
Primary tumor (pT)
- TX: primary tumor cannot be assessed
- T1: tumor invades the mucosa or submucosa only and is ≤ 1 cm (duodenal tumors); tumor ≤ 1 cm and confined within the sphincter of Oddi (ampullary tumors)
- T2: tumor invades the muscularis propria or is ≥ 1 cm (duodenal); tumor invades through sphincter into duodenal submucosa or muscularis propria or is ≥ 1 cm (ampullary)
- T3: tumor invades the pancreas or peripancreatic adipose tissue
- T4: tumor invades the visceral peritoneum (serosa) or other organ
- Notes: Tis is not a valid category under AJCC 8th edition staging for these tumors
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 include duodenal, hepatic, pancreatoduodenal, infrapyloric, gastroduodenal, pyloric, superior mesenteric and pericholedochal nodes
Stage grouping
- 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
Registry data collection variables
- Size of tumor
- Maximum depth of invasion
- Number of tumors
- Lymph node status
- Grade
- Mitotic count
- Ki67 labeling index
- Perineural invasion
- Lymphovascular invasion
- Margin status
- Functional status
- Genetic syndrome
- Location in duodenum
- Type of surgery
- Preoperative chromogranin A level
- Preoperative pancreastatin level
- Preoperative neurokinin level
- Age of patient
- Histologic variants
Histologic grade
- Grading is not formally part of the staging system
- Most pathologists use the ENETS / WHO grading criteria:
- G1: mitotic rate < 2 per 10 high power fields and Ki67 rate < 3%
- G2: mitotic rate 2 - 20 per 10 high power fields or Ki67 rate 3 - 20%
- G3: mitotic rate > 20 per 10 high power fields or Ki67 rate > 20%
Board review style question #1
Regarding neuroendocrine tumors of the ampulla, which T category in the 8th edition AJCC staging guide includes size as a factor?
- T1 only
- T1 and T2
- T1, T2, and T3
- T2, T3, and T4
Board review style answer #1
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