Notes by dr Claudio Italiano
The carcinoma of the ampulla of Vater is a malignant tumor that at the time of diagnosis has dimensions of 2-2.5 cm and is found at the end of the common part of the common bile duct, where it passes through the duodenum wall and the papilla of Vater .
The common bile duct joins this level with the Wirsung and
flows through the vater's ampulla into the duodenum. The extreme distal part of
the common bile duct is dilated (i.e., forms the vater ampoule) and is
surrounded by the sphincter of Oddi which coils itself around the terminal
portion of the duct. Following bile duct obstruction, carcinoma manifests early,
unlike pancreatic cancer which is often late agnostic. Surgical resection is
the only long-term curative therapeutic option.
Surgical or radiological
decompression allows to resolve the obstruction and to give adequate control to
the pain and, therefore, a good quality of life, but does not extend the
survival rate. Patofiosis: 90% of ampullary tumors are adenocarcinomas.
Neuroendocrine tumors, cystadenomas and adenomas are uncommon histological
types. Tumors originate from ductal epithelial cells and generally invade the
parenchyma of the pancreas. In the more advanced states of the disease the
peripancreatic tissue and the adventitia and the vessels as well as the portal
and superior mesenteric veins may be affected.
Lymph node metastasis is present in half of the patients. Pericanalicular lymph nodes are those that are first affected by the process.
The lymph nodes around
the upper mesenteric, splenic, gastroduodenal, common hepatic arteries and the
celiac trunk itself constitute the secondary stations involved. Perineural,
lymphatic and vascular invasion are associated with an unfavorable prognosis.
The liver is the most common site (66%) site of metastasis, followed by lymph
nodes (22%). In more advanced cases, lung metastases may also occur
In the USA: The carcinoma of the ampulla of Vater is a little common tumor;
just over 2,000 cases are diagnosed each year. The cancer of the ampoule, so,
represents only 0.2% of all malignant tumors of the gastric tract and about 7%
of periampullar carcinomas. Adenocarcinoma of the ampoule of Vater is the
second most common periampullar tumor. We do not know the global incidence.
Most of these neoplasms are resectable and therefore curable already at the
time of diagnosis, but the 5-year survival is only 40%. Preoperative mortality
has significantly reduced in the last decade, thanks to anesthesia, imaging
techniques and post-operative management. However, the percentage of surgical
morbidity remains high, with a range of 25-65% even in centers with expert
staff. Pancreatic fistulas, prolonged gastric emptying, surgical complications
such as ferrite and ulceration are the most frequent complications.
Post-operative mortality is in the best centers between 2-5%.
Race:
He does not seem to prefer any race.
Sex: same
Age:
Ampoule cancer seems to be more frequent between 50 and 70 years of age.
Patient with percutaneous drainage of the hepatic
ducts, for palliation of neoplastic obstructive jaundice
Jaundice: it is the condition with which the disease occurs in 3/4 of cases;
Ampoule cancer has no other early symptoms. Jaundice can also be intermittent if
the obstruction and intraductal pressure are reduced and the bile makes its way
through the necrosis of the central tissues of the ampoule.
Other characteristics:
Itching, loss of appetite, dyspepsia and vomiting; these may be present if the
duodenal lumen is compromised, progressive weight loss, epigastric pain, or pain
in the right hypochondrium. Back pain appears, unfortunately, in advanced stages;
pancreatic cholera is characterized by diarrhea and is due to the absence of
lipase inside the lumen, caused by the ductal obstruction. Hematemesis, melena
and hematochezia are uncommon signs caused by the bleeding of the tumor.
The sign of Courvoisier, with jaundice without pain can be associated with a
palpable gallbladder. However, it must be said that this sign may also depend on
a calculus obstruction of the common duct which determines the distention of the
gallbladder. Fever can occur if an ascending cholangitis is determined. Even an
hepatomegaly can manifest itself. It is rare but it is possible that patients
also have acute pancreatitis or a migrating thrombophlebitis. An epigastric mass
or of the palpable lymph nodes in the supraclavicular site are signs of advanced
disease and, therefore, of operable tumor.
Etiology is poorly understood. Patients with familial polyposis have an
increased risk of developing both diseases, ie the good and malignant tumors of
the ampoule. (Burke, 1999). More than 50-90% of subjects with FAP develop
duodenal adenoma, mainly located around the papilla (Griffioen, 1998).
Abnormal genomics can determine it. (Scarpa and Zamboni, 1999); K-ras mutations
can determine it (Berndt, 1998).
- Blood chemistry tests:
loss of anemia due to bleeding of the ampullary mass; hyperbilirubinemia of the
conjugated or direct type for jaundice, due to the blockage of the biliary flow;
increase in cholestasis enzymes, including gammaGT and alkaline phosphatase for
blockade of bile outflow and pressure increase in the cholecode, resulting in
enzyme synthesis. Transaminase release, including ALT or alanine
aminotransferase and AST or aspartate aminotransferase, if the obstruction is
prolonged.
The test for occult blood in the stool may be positive if there is a
bleeding tumor. In cases of complete bloody obstruction, faeces may be pale,
cretaceous, so-called hypochic feces or silvery feces.
There may also be an
increase in amylasemia which is not uncommon. Alteration of the coagulation
profile with increased prothrombin time and bleeding prolongation and
coagulation values. The urine has bile pigments, there is no urinary
urobilinogens which means a complete obstruction, the tumor markers are not
specific enough: CA 19-9 is the most studied and sensitive, but unfortunately
has little predictive value in ampullary carcinoma . Other more sensitive
markers are:
CEA (carcinoembriogenetic antigen); DU-PHAN-2, alpha-fetoprotein and pancreatic
oncofetal antigen or POA.
- Other articles on cancer
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