Ampulla of Vater
Carcinoma
Adenocarcinoma

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

Revised: 13 August 2018, last major update August 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: ampulla adenocarcinoma
Cite this page: Gulwani, H. Adenocarcinoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/ampullaadenocarcinoma.html. Accessed November 18th, 2018.
Definition / 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 coexisting 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)
Diagrams / tables

Images hosted on other servers:

KRAS mutations

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 70s, 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, periampullary or mixed
  • Often small
  • Common bile duct often dilated
Gross images

Images hosted on PathOut server:

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

Various images



Images hosted on other servers:

Mass with central ulceration

Microscopic (histologic) 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
  • Noninvasive 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
Microscopic (histologic) images

Images hosted on PathOut server:

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

H&E


CEA

CK7

CK20

MSH2



CDX2

Chromogranin

Synaptophysin



Images hosted on other servers:

Intestinal, pancreaticobiliary and other subtypes

Various images


Early tumor

Budding

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