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 sysytem.[1]
Staging
According to The American Joint Committee on Cancer (AJCC), there are four stages of carcinoid syndrome based on the TNM staging sysytem.[1]
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.
|
References
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