Ampulla of Vater
Carcinoma
Adenocarcinoma

Author: Hanni Gulwani, M.D. (see Authors page)

Revised: 2 March 2016, last major update August 2014

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: ampulla adenocarcinoma
General
  • By definition, centered in ampulla of Vater, arises from ampullary intestinal mucosa
  • If advanced, cannot distinguish tumor origin between ampulla, distal common bile duct or pancreas
Etiology
  • May arise from villous adenoma or villoglandular polyp; usually is a co-existing adenoma
  • Carcinomas of pancreas, gallbladder, extrahepatic bile ducts and ampulla have common embryonic cellular ancestry, differentiation pathways, mucosal histologic patterns and population-related tumor development, indicating a field effect in carcinogenesis
  • Carcinomas of pancreas are more common than at these other sites because pancreatic ductal system has a greater surface area (Arch Pathol Lab Med 2009;133:67)
Sites
  • May arise within ampulla (intra-ampullary) or in periampullary duodenum without significant ampullary involvement
  • Site of origin may be difficult to determine
Clinical Features
  • 5% of GI malignancies are ampullary or periampullary adenocarcinoma
  • 80% of ampullary neoplasms and 90% of ampullary malignancies are adenocarcinomas
  • Peak age in 70’s, men affected more than women
  • 1/3 have other malignancies
  • May be associated with multiple polyposis syndrome and neurofibromatosis
  • Causes jaundice, abdominal pain, occasionally pancreatitis
  • Nodal metastases at diagnosis in 35% (usually adjacent periampullary nodes)
  • Immunohistochemically detected lymph node micrometastasis indicates intensive lymphatic spread, and adversely affects the survival of patients with ampullary carcinoma (World J Surg 2006;30:985)
  • Metastasizes to liver, lung, peritoneum, pleura
  • On CT scan, a small intra-ampullary tumor may show dilated ducts without a mass; ultrasound may be better imaging modality
Prognostic Factors
Case Reports
Treatment
  • Whipple procedure
Gross Description
  • Tumor bulges into duodenal lumen, may be intra-, peri-ampullary or mixed
  • Often small
  • Common bile duct often dilated
Gross Images

Images hosted on Pathout server:

Contributed by Dr. Semir Vranic, University of Sarajevo, Bosnia and Herzegovina



Images hosted on other servers:

Mass with central ulceration

Micro Description
  • Usually poorly differentiated, may have papillary component resembling villous adenoma or villoglandular polyp
  • Intestinal (arising from covering intestinal mucosa of papilla, large elongated tubules) or pancreaticobiliary (arise from ductal epithelium that penetrates duodenal muscularis propria, smaller glands/tubules with desmoplastic stroma)
  • 50% have vascular invasion, occasional perineural invasion
  • Non-invasive papillary lesions resemble colorectal villous adenoma; may be associated with familial colonic polyposis with a high risk for malignant transformation
  • Differentiation is based on % glands (well: >95%, moderate: 50-95%, poor: 5-49%, undifferentiated: <5%)
  • Lamina propria invasion is considered by some as invasion due to rich lymphatics (Am J Surg Pathol 1991;15:1188)
  • Also PanIN (1/3, Mod Pathol 2001;14:139), pancreatitis (1/3)
  • Variants: colloid, hepatoid, medullary, micropapillary, mixed acinar-endocrine, mucinous, neuroendocrine, Paneth cell, signet ring cell
Micro Images

Images hosted on Pathout server:

Contributed by Dr. Semir Vranic, University of Sarajevo (Bosnia and Herzegovina)


Line 1: H&E; Line 2: CEA, CK7, CK20, MSH2




Line 2: CDX2 (left); chromogranin (middle); synaptophysin (right)



Images hosted on other servers:

Intestinal, pancreaticobiliary and other subtypes

Various images


Early tumor

Budding



Images hosted on Nature.com:

Various images

Early tumor


CDX2 staining: various images


Missing Image

DPC4 staining in invasive carcinoma and
adenomas with high grade dysplasia


Positive Stains
Negative Stains
Molecular / Cytogenetics Description
Differential Diagnosis
  • Adenomatous changes in submucosal glands / ductules simulating invasion, cautery artifact, reactive epithelial atypia (Am J Clin Pathol 2009;132:506)
  • Common bile duct carcinoma: thickening of common bile duct, granular mucosa, usually well differentiated adenocarcinoma formed by small glands with marked desmoplasia
  • Duodenal adenocarcinoma-not ampullary: may also arise in villous adenoma
Additional References