Carcinoid syndrome staging

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Parminder Dhingra, M.D. [2]

Overview

Staging

Definitions of TNM

The American Joint Committee on Cancer (AJCC) has designated staging by TNM classification to define neuroendocrine tumors:[1]

Neuroendocrine Tumors: Stomach

T Primary tumor
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
Tis Carcinoma in situ/dysplasia (tumor size <0.5 mm), confined to mucosa
T1 Tumor invades lamina propria or submucosa and ≤1 cm in size
T2 Tumor invades muscularis propria or >1 cm in size
T3 Tumor penetrates subserosa
T4 Tumor invades visceral peritoneum (serosal) or other organs or adjacent structures
For any T, add (m) for multiple tumors


N Regional Lymph Nodes
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis


M Distant Metastases
M0 No distant metastases
M1 Distant metastasis

Neuroendocrine Tumors: Duodenum/Ampulla/Jejunum/Ileum

T Primary tumor
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor invades lamina propria or submucosa and size ≤1 cmb (small intestinal tumors); tumor ≤1 cm (ampullary tumors)
T2 Tumor invades muscularis propria or size >1 cm (small intestinal tumors); tumor >1 cm (ampullary tumors)
T3 Tumor invades through the muscularis propria into subserosal tissue without penetration of overlying serosa (jejunal or ileal tumors) or invades pancreas or retroperitoneum (ampullary or duodenal tumors) or into nonperitonealized tissues
T4 Tumor invades visceral peritoneum (serosa) or invades other organs
For any T, add (m) for multiple tumors


N Regional Lymph Nodes
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis


M Distant Metastases
M0 No distant metastases
M1 Distant metastasis

Neuroendocrine Tumors: Colon or Rectum

T Primary tumor
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor invades lamina propria or submucosa and size ≤2 cm
T1a Tumor size <1 cm in greatest dimension
T1b Tumor size 1–2 cm in greatest dimension
T2 Tumor invades muscularis propria or size >2 cm with invasion of lamina propria or submucosa
T3 Tumor invades through the muscularis propria into the subserosa or into nonperitonealized pericolic or perirectal tissues
T4 Tumor invades peritoneum or other organs
For any T, add (m) for multiple tumors


N Regional Lymph Nodes
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis


M Distant Metastases
M0 No distant metastases
M1 Distant metastasis

Appendiceal Carcinoids

T Primary tumor
TX Primary tumor cannot be assessed
T0 No evidence of primary tumor
T1 Tumor ≤2 cm in greatest dimension
T1a Tumor ≤1 cm in greatest dimension
T1b Tumor >1 cm but not >2 cm
T2 Tumor >2cm but not >4 cm or with extension to the cecum
T3 Tumor >4 cm or with extension to the ileum
T4 Tumor directly invades other adjacent organs or structures, e.g., abdominal wall and skeletal muscle


N Regional Lymph Nodes
NX Regional lymph nodes cannot be assessed
N0 No regional lymph node metastasis
N1 Regional lymph node metastasis


M Distant Metastases
M0 No distant metastases
M1 Distant metastasis

Anatomic Stage/Prognostic Groups

Stomach, Duodenum/Ampulla/Jejunum/Ileum, and Colon or Rectum

Stage T N M
0 Tis N0 M0
I T1 N0 M0
IIA T2 N0 M0
IIB T3 N0 M0
IIIA T4 N0 M0
IIIB Any T N1 M0
IV Any T Any N M1

Appendiceal Carcinoids

Stage T N M
I T1 N0 M0
II T2, T3 N0 M0
III T4 N0 M0
Any T N1 M0
IV Any T Any N M1

Residual Tumor (R)

Carcinoma and Carcinoid
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.


References

  1. Stage Information for GI Carcinoid Tumors . http://www.cancer.gov/types/gi-carcinoid-tumors/hp/gi-carcinoid-treatment-pdq#section/_163 (date). September 21, 2015

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