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.[1]

  • 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

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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