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

According to The American Joint Committee on Cancer (AJCC), there are four stages of carcinoid syndrome based on the TNM staging system.

Staging

According to The American Joint Committee on Cancer (AJCC), there are four stages of carcinoid syndrome based on the TNM staging system.

• Proliferative rate as assessed by mitotic count and Ki-67 labeling index, is of prognostic significance in well-differentiated neuroendocrine tumors independent of tumor stage.

TNM Classification

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 cm (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, 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 Carcinoid

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

Lung Carcinoid

T	Primary tumor
TX	Primary tumor can’t be assessed or cancer cells were seen on sputum cytology or bronchial washings but no tumor can be found
T0	No evidence of a primary tumor
Tis	Tumor is found only in the top layers of cells lining the air passages. It has not invaded into deeper lung tissues. This stage is also known as carcinoma in situ
T1	Tumor is no larger than 3 centimeters (cm) – slightly less than 1¼ inches – across. It has not reached the membranes that surround the lungs (visceral pleura), and it does not affect the main branches of the bronchi
T1a	Tumor is 2 cm (about 4/5 of an inch) or less across
T1b	Tumor is larger than 2 cm but not larger than 3 cm across
T2	Tumor has 1 or more of the following features:
	Tumor is larger than 3 cm but not larger than 7 cm across
T2a	Tumor is 5 cm or less across but still larger than 3 cm
T2b	Tumor is larger than 5 cm across but not larger than 7 cm
T3	Tumor has 1 or more of the following features:
	Tumor is larger than 7 cm across
	Tumor has grown into the chest wall, the breathing muscle that separates the chest from the abdomen (diaphragm), the membranes surrounding the space between the lungs (mediastinal pleura), or membranes of the sac surrounding the heart (parietal pericardium)
	Tumor invades a main bronchus and is closer than 2 cm (about ¾ inch) to the carina, but it does not involve the carina itself
	Tumor has grown into the airways enough to cause an entire lung to collapse or to cause pneumonia in the entire lung
	Two or more separate tumor nodules are present in the same lobe of a lung
T4	The cancer has 1 or more of the following features:
	Tumor of any size has grown into the mediastinum, heart, aorta, windpipe, esophagus, vertebrae, or the carina
	Presence of two or more separate tumor nodules in different lobes of the same lung

N	Regional Lymph Nodes
NX	Regional lymph nodes cannot be assessed
N0	No regional lymph node metastasis
N1	Metastasis to lymph nodes within the lungs and/or hilar lymph nodes. Metastasis on the ipsilateral side
N2	Metastasis to lymph nodes around the carina or to lymph nodes in the mediastinum on the ipsilateral side
N3	Metastasis to bilateral lymph nodes, and/or spread to hilar or mediastinal lymph nodes on the contralateral side

M	Distant Metastases
M0	No distant metastases
M1a	Any of the following:
	The cancer has spread to the other lung
	Cancer cells are found in the fluid around the lung (called a malignant pleural effusion)
	Cancer cells are found in the fluid around the heart (called a malignant pericardial effusion)
M1b	The cancer has spread to distant lymph nodes or to organs such as the liver, bones, or brain

Anatomic Stage

Stomach, Duodenum/Ampulla/Jejunum/Ileum, and Colon/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 Carcinoid

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

Lung Carcinoid

Stage	T	N	M
0	Tis	N0	M0
IA	T1	N0	M0
IB	T2a	N0	M0
IIA	T1	N1	M0
	T2a	N1	M0
	T2b	N0	M0
IIB	T2	N1	M0
	T3	N0	M0
IIIA	T1 to T3	N2	M0
	T3	N1	M0
	T4	N0 or N1	M0
IIIB	Any T	N3	M0
	T4	N2	M0
IV	Any T	Any N	M1a
	Any T	Any N	M1b

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