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Appendix

Other tumors

Staging

Reviewer: Jaleh Mansouri, M.D. (see Reviewers page)
Revised: 21 November 2012, last major update August 2012
Copyright: (c) 2003-2012, PathologyOutlines.com, Inc.

General
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● In AJCC 7th edition, appendiceal carcinomas have a separate staging system from colorectal tumors
● There are separate staging systems for appendiceal carcinoma and appendiceal carcinoid tumors

Appendiceal carcinoma
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Primary tumor (T)
● TX: Primary tumor cannot be assessed
● T0: No evidence of primary tumor
● Tis: Carcinoma in situ: intraepithelial or invasion of lamina propria, but not through muscularis mucosa into submucosa
● T1: Tumor invades submucosa
● T2: Tumor invades muscularis propria
● T3: Tumor invades through the muscularis propria into the subserosa or into mesoappendix
● T4: Tumor penetrates visceral peritoneum, including mucinous peritoneal tumor within the right lower quadrant, or directly invades other organs or structures
● T4a: Tumor penetrates visceral peritoneum, including mucinous peritoneal tumor within the right lower quadrant
● T4b: Tumor directly invades other organs or structures

Notes:
● Direct invasion in T4 includes invasion of other segments of the colorectum by way of the serosa, e.g., invasion of ileum
● Tumor that is adherent to other organs or structures grossly is classified cT4b; however, if no tumor is present in the adhesion, the classification should be pT1-3 depending on the anatomical depth of wall invasion

Regional lymph nodes (N)
● NX: Regional lymph nodes cannot be assessed
● N0: No regional lymph node metastasis
● N1: Metastasis in 1-3 regional lymph nodes
● N2: Metastasis in 4 or more regional lymph nodes

Notes:
● A satellite peritumoral nodule or tumor deposit in the periappendiceal adipose tissue of a primary carcinoma without histologic evidence of residual lymph node in the nodule may represent discontinuous spread (T3), venous invasion with extravascular spread (T3, V1/2) or a totally replaced lymph node (N1/2)
● Replaced nodes should be counted as positive nodes, while discontinuous spread or venous invasion should be counted in the site-specific factor tumor deposit
● Examination should include 12 or more nodes, but classify as pN0 if <12 nodes but all are negative

Distant Metastasis (M)
● M0: No distant metastasis
● M1: Distant metastasis
● M1a: Intraperitoneal metastasis beyond the right lower quadrant, including pseudomyxoma peritonei
● M1b: Nonperitoneal metastasis

● G1: Well differentiated or mucinous low grade
● G2: Moderately differentiated or mucinous high grade
● G3: Poorly differentiated or mucinous high grade
● G4: Undifferentiated


Table - Appendiceal carcinoma

Appendiceal carcinoid
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Primary tumor (T)
● TX: Primary tumor cannot be assessed
● T0: No evidence of primary tumor
● T1: Tumor 2 cm or less in greatest dimension
● T1a: Tumor 1 cm or less in greatest dimension
● T1b: Tumor more than 1 cm but not more than 2 cm
● T2: Tumor more than 2 cm but not more than 4 cm or with extension to the cecum
● T3: Tumor more than 4 cm or with extension to the ileum
● T4: Tumor directly invades other adjacent organs or structures; e.g. abdominal wall and skeletal muscle

Notes:
● Tumor that is adherent to other organs or structures grossly is classified cT4; however, if no tumor is present in the adhesion, the classication should be pT1-3
● Penetration 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

Regional lymph nodes (N)
● NX: Regional lymph nodes cannot be assessed
● N0: No regional lymph node metastasis
● N1: Regional lymph node metastasis

Distant Metastasis (M)
● M0: No distant metastasis
● M1: Distant metastasis

● G1: Mitotic rate <2 per 10 HPF; Ki-67 index < 2%
● G2: Mitotic rate 2 to 10 per 10 HPF; Ki-67 index 3 to 20%
● G3: Mitotic rate >10 per 10 HPF; Ki-67 index > 20%

Notes:
● pM0 does not exist
● Examination should include 12 or more nodes, but classify as pN0 if <12 nodes but all are negative
● Mitotic rate based on counting 50 high-power fields (40x objective) in areas of highest activity

Stage grouping
● Stage I: T1 N0 M0
● Stage II: T2-3 N0 M0
● Stage III: T4 N0 M0 or Any T N1 M0
● Stage IV: Any T Any N M1

End of Appendix > Other tumors > Staging

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