^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
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.
T4	For any T, add (m) for multiple tumors.

Table 3. Regional Lymph Nodes (N)^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
NX	Regional lymph nodes cannot be assessed.
N0	No regional lymph node metastasis.
N1	Regional lymph node metastasis.

Table 4. Distant Metastases (M)^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
M0	No distant metastases.
M1	Distant metastasis.

Neuroendocrine Tumors: Duodenum/Ampulla/Jejunum/Ileum

Table 5. Primary Tumor (T)^a

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TX	Primary tumor cannot be assessed.
T0	No evidence of primary tumor.
T1	Tumor invades lamina propria or submucosa and size ≤1 cm^b (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.
T4	For any T, add (m) for multiple tumors.

^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
^bTumor limited to ampulla of Vater for ampullary gangiocytic paraganglioma.

Table 6. Regional Lymph Nodes (N)^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
NX	Regional lymph nodes cannot be assessed.
N0	No regional lymph node metastasis.
N1	Regional lymph node metastasis.

Table 7. Distant Metastases (M)^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
M0	No distant metastases.
M1	Distant metastasis.

Neuroendocrine Tumors: Colon or Rectum

Table 8. Primary Tumor^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
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.
T4	For any T, add (m) for multiple tumors.

Table 9. Regional Lymph Nodes (N)^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
NX	Regional lymph nodes cannot be assessed.
N0	No regional lymph node metastasis.
N1	Regional lymph node metastasis.

Table 10. Distant Metastases (M)^a

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^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
M0	No distant metastases.
M1	Distant metastasis.

Table 11. Anatomic Stage/Prognostic Groups for Stomach, Duodenum/Ampulla/Jejunum/Ileum, and Colon or Rectum^a

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Stage	T	N	M
^aReprinted with permission from AJCC: Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
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

A new classification is added for carcinoid tumors that were not classified previously by TNM. This is a new classification. There are substantial differences between the classification schemes of appendiceal carcinomas and carcinoids and between appendiceal carcinoids and other well-differentiated gastrointestinal neuroendocrine tumors (carcinoids).[2]

Serum chromogranin A is identified as a significant prognostic factor.[2]

Table 12. Primary Tumor (T)^a,b

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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.^c

^aReprinted with permission from AJCC: Appendix. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 133-41.
^bTumor that is adherent to other organs or structures, grossly, is classified cT4. However, if no tumor is present in the adhesion, microscopically, the classification should be classified pT1–3 depending on the anatomical depth of wall invasion.
^cPenetration of the mesoappendix does not seem to be as important a prognostic factor as the size of the primary tumor and is not separately categorized.

Table 13. Regional Lymph Nodes (N)^a

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^aReprinted with permission from AJCC: Appendix. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 133-41.
NX	Regional lymph nodes cannot be assessed.
N0	No regional lymph node metastasis.
N1	Regional lymph node metastasis.

Table 14. Distant Metastasis^a

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^aReprinted with permission from AJCC: Appendix. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 133-41.
M0	No distant metastasis.
M1	Distant metastasis.

pTNM Pathologic Classification. The pT, pN, and pM categories correspond to the T, N, and M categories except that pM0 does not exist as a category.[2]

pN0. Histological examination of a regional lymphadenectomy specimen will ordinarily include 12 or more lymph nodes. If the lymph nodes are negative, but the number ordinarily examined is not met, classify as pN0.[2]

Table 15. Anatomic Stage/Prognostic Groups^a

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Carcinoid
^aReprinted with permission from AJCC: Appendix. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 133-41.
Stage	T	N	M
I	T1	N0	M0
II	T2, T3	N0	M0
III	T4	N0	M0
III	Any T	N1	M0
IV	Any T	Any N	M1

Carcinoid. Histologic grading is not carried out for carcinoid tumors, but a mitotic count of 2–10 per 10 hpf and/or focal necrosis are features of atypical carcinoids (well-differentiated neuroendocrine carcinomas), a type seen much more commonly in the lung than in the appendix.[2]

Goblet cell carcinoids are classified according to the carcinoma scheme.[2]

This staging classification applies to carcinoids that arise in the appendix. The histologic types include the following:[2]

Carcinoid tumor.
Well-differentiated neuroendocrine tumor.
Tubular carcinoid.
Goblet cell carcinoid.
Adenocarcinoid.
Atypical carcinoid.

Well-differentiated neuroendocrine carcinoma after resection (relevant to resection margins that are macroscopically involved by tumor).[2]

Table 16. Residual Tumor (R)^a

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Carcinoma and Carcinoid
^aReprinted with permission from AJCC: Appendix. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 133-41.
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

Neuroendocrine tumors. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 181-9.
Appendix. In: Edge SB, Byrd DR, Compton CC, et al., eds.: AJCC Cancer Staging Manual. 7th ed. New York, NY: Springer, 2010, pp 133-41.

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