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]

Staging

Definitions of TNM

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

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.