Review
Copyright ©The Author(s) 2018.
World J Gastrointest Oncol. Nov 15, 2018; 10(11): 370-380
Published online Nov 15, 2018. doi: 10.4251/wjgo.v10.i11.370
Table 1 Proposed immunohistochemical markers for ampulla of Vater carcinoma histological classification (adapted from Mafficini et al[16])
Immunohistochemical marker criteria presentIntestinal typePancreatobiliary type positiveMixed/Ambiguous typeNote
Ang et al[12] (MUC1, MUC2, CDX2, CK20)Positive CK20 or CDX2 or MUC2, and negative MUC1 Positive CK20 and CDX2, and MUC2 and any MUC1Positive MUC1 and negative CDX2, and negative MUC2 and any CK20All other combinations
Chang et al[13] (MUC1, CDX2)Positive CDX2 or negative MUC1Negative CDX2 and positive MUC1Not applicableCDX2 positivity based on H score (percentage of positive cells × intensity of staining) > 35 MUC1 positivity based on any staining
Gingras et al[17] (MUC1, CDX2)Ratio of the CDX2/MUC1 H score ≥ 2Ratio of the CDX2/MUC1 H score < 0.5Ratio of CDX2/MUC1 H score ≥ 0.5 and < 2Use only MUC1 and CDX2 as per Chang et al[13], with H scores for both CDX2 and MUC1
Mafficini et al[16] (MUC1, MUC2, CDX2, CK20)Positive CK20 or CDX2 or MUC2, and negative MUC1Positive MUC1 and negative CDX2, and negative MUC2 and any CK20All other combinations
Table 2 Frequency of significantly mutated ampulla of Vater carcinoma genes in different histotypes and compared to colorectal and pancreatic adenocarcinoma (adapted from Yachida et al[14])
Yachida et al[14]
Gingras et al[17]
Biankin et al[36] Pancreatic carcinoma, %Colorectal Carcinoma (TCGA), %[35]
Intestinal type, %Pancreato-biliary type, %Mixed type, %Pancreato-biliary type, %Intestinal type, %
APC (50)KRAS (68)KRAS (50)TP53 (72)TP53 (65)KRAS (99)APC (81)
TP53 (39)TP53 (67)APC (50)KRAS (65)KRAS (46)TP53 (33)TP53 (60)
KRAS (39)SMAD4 (20)TP53 (41)SMAD4 (18)APC (41)SMAD4 (16)KRAS (43)
CTNNB1 (26)CTNNB1 (15)SMARCA4 (27)CDKN2A (16)PIK3CA (26)MLL3 (7)TTN (31)
ARID2 (18)ERBB3 (14)PIK3CA (23)PIK3CA (13)SMAD4 (20)ATM (5)PIK3CA (18)
ERBB2 (14)GNAS (12)SMAD 4 (23)ARID1A (13)TGFBR2 (17)NALCN (5)FBXW7 (14)
ACVR2A (13)CDH10 (12)SOX 9 (23)APC (11)ARID2 (17)ARID1A (4)SMAD4 (10)
SMAD4 (13)ELF3 (11)CDKN2A (23)ATM (10)ELF3 (7)SF3B1 (4)NRAS (9)
GNAS (13)CDKN2A (9)ARID1A (18)TGFBR2 (10)CTNNB1 (17)TGFBR2 (4)TCF7L2 (9)
SOX9 (13)TGFBR2 (14)FBXW7 (8)NF1 (15)ARID2 (3)FAM123B (7)
Table 3 Ampulla of Vater cancer staging AJCC 2017[21]
Primary tumor (T)
T categoryT criteria
TXPrimary tumor cannot be assessed
T0No evidence of primary tumor
TisCarcinoma in situ
T1Tumor limited to ampulla of Vater or sphincter of Oddi, or tumor invades beyond the sphincter of Oddi (perisphincteric invasion) and/or into the duodenal submucosa
T1aTumor limited to ampulla of Vater or sphincter of Oddi
T1bTumor invades beyond the sphincter of Oddi (perisphincteric invasion) and/or into the duodenal submucosa
T2Tumor invades into the muscularis propria of the duodenum
T3Tumor directly invades the pancreas (up to 0.5 cm) or tumor extends more than 0.5 cm into the pancreas, or extends into peripancreatic or periduodenal tissue or duodenal serosa without involvement of the celiac axis or superior mesenteric artery
T3aTumor directly invades pancreas, up to 0.5 cm
T3bTumor extends more than 0.5 cm into the pancreas, or extends into peripancreatic tissue or duodenal serosa without involvement of the celiac axis or superior mesenteric artery
T4Tumor involves the celiac axis, superior mesenteric artery, and/or common hepatic artery, irrespective of size
Regional lymph nodes (N)
N categoryN criteria
NXRegional lymph nodes cannot be assessed
N0No regional lymph node metastasis
N1Metastasis to 1 to 3 regional lymph nodes
N2Metastasis in > 3 regional lymph nodes