Notes by dr Claudio Italiano
Before proceeding with the resection of a primary tumor, especially if localized to the lung, the gastrointestinal tract or the breast, the presence of a metastatic invasion of the liver must be carefully researched.
A presumptive diagnosis may be based on evidence of abnormal liver function
tests, especially increased alkaline phosphatase, or demonstration of
hepatic mass by hepatic scintigraphy, ultrasound, CT or MRI. Blind
percutaneous hepatic biopsy may be diagnostic only in 60-80% of hepatomegaly
cases and elevated levels of alkaline phosphatase. The examination of
repeated sections of the biopsy samples, two or three repetitions of the
biopsy or the cytological examination of the aspirated liver material can
increase the diagnostic possibilities by 10-15%. These are significantly
greater when biopsies are performed under ultrasound or CT scan, or during
laparoscopy.
Most liver metastases respond poorly to all forms of therapy, which usually
have only palliative character. In special cases, a large but single
metastasis can be removed surgically. Systemic chemotherapy may slow the
growth of the tumor for a short time, but it does not change the prognosis
appreciably.
Chemoembolization, intrahepatic chemotherapy and alcoholization may be palliative therapeutic approaches. It remains to be established whether drugs, associations of different chemotherapeutics or new strategies (including immunological labeling) will prove to be more effective.
Benign tumors of the extracipatic bile ducts very rarely cause mechanical
biliary obstruction. In most cases they are papillomas, adenomas or cystadenomas
that manifest themselves with obstructive jaundice and emobilia. The
adenocarcinoma of extrahepatic ducts is more common: it affects male sex more
frequently (60% of cases) and has a maximum incidence between the fifth and
seventh decade of life. The factors that seem to predispose to the onset of this
tumor are the following:
1) some chronic hepatobiliary parasitic infections;
2) congenital anomalies with ectatic ducts;
3) sclerosing cholangitis and ulcerative colitis;
4) occupational exposure to carcinogens of the biliary tract (rubber workers and
car production workers). However, it has not been shown with certainty that
cholelithiasis is a predisposing factor to cholangiocarcinoma.
The blood tests that are altered in these cases are the cholestasis indices (bilirubin,
gamma-GT and alkaline phosphatase). The increase in transaminases is less
frequent. The most commonly used tumor marker is Ca 19-9. Blood tests may offer
help, but it is essential to obtain images of the liver with abdominal
ultrasound which is the first instrumental examination to be performed and
possibly in a second phase the TAC and the Colangio-RMN which allow a detailed
assessment of the pathways. bile.
Based on the site of origin, the tumors of the biliary route are divided into:
a) Klatskin's tumors (born to the hilum of the liver, right at the exit of the
biliary tract from the liver)
b) intrahepatic tumors (born from the biliary pathway within the liver);
c) extrahepatic tumors (born from the biliary pathway outside the liver).
Depending on the location of origin, the diagnosis can be made at different times and this can also change the prognosis. Generally the prognosis is better for extrahepatic tumors that give signs immediately.
This tumor can be widespread or nodular; the nodular form often occurs at the bifurcation of the common bile duct (Klatskin's tumors). This tumor is usually associated with collapsed gallbladder and this allows visualization of proximal bile ducts with cholangiography. Patients with cholangiocarcinoma usually arrive at clinical observation for the appearance of biliary obstruction, jaundice accompanied by pain, itching, weight loss and acetic stool; in some cases there is also a dull pain, vaguely localized to the upper right abdominal quadrant.
The finding of hepatomegaly and a palpable and distended gallbladder is frequent (unless the lesion affects a high portion of the duct). Fever is uncommon in the absence of ascending cholangitis. Because biliary obstruction occurs gradually, cholangiocarcinoma is usually already in a very advanced stage when it manifests clinically. In most cases the neoplasm is diagnosed by cholangiography after ultrasound examination of intrahepatic bile ducts dilatation. Any focal narrowing of the bile ducts should probably be considered malignant until proven otherwise. In some cases treated with palliative drainage (stent placement) of the biliary tree a survival of 1-2 years has been achieved.
to know more>> The carcinoma of the Vater's ampulla
The papilla of Vater may be affected by the extension of tumors that originate in other portions of the duodenum or may itself be the site of origin of sarcomas, carcinoids or adenocarcinomas.
The vegetal adenocarcinomas are characterized by slow growth and a more favorable clinical prognosis than infiltrative and diffuse papilla tumors, which are more often invasive.
The clinical presentation is usually that of an obstructive jaundice. In the suspicion of a carcinoma of the papilla of Vater, the best diagnostic technique is probably the endoscopic retrograde cannulation of the pancreatic duct (endoscopic retrograde cholangiopancreatography) that allows both direct endoscopic inspection and papillary biopsy and the execution of pancreatography for exclude the diagnosis of malignant neoplasm of the pancreas.
The treatment of Vater's papilla carcinoma consists of a wide and often radical surgical exeresis. In about 20% of cases there are already lymph node or distant metastases at the time of surgery and the 5-year survival in this group of patients undergoing surgery is only 5-10%. In contrast, in the absence of metastasis, radical pancreaticoduodenectomy (Whipple technique) allows a 5-year survival of up to 40%, with several cases of survivors also in the long term.
Most gallbladder tumors develop in the presence of calculations rather than polyps. In patients with gallbladder lithiasis the risk of developing gallbladder cancer, even if increased, is always very low. In fact, in a clinical study, gallbladder cancer developed only in 5 out of 2583 patients with gallbladder lithiasis followed on average for 13 years. In the United States, adenocarcinomas account for most of the 6500 new gallbladder cancer cases diagnosed each year. The male / female ratio is 1: 4 and the average age at diagnosis is 70 years.
The clinical presentation is characterized, in most cases, by continuous pain in the right hypochondrium associated with weight loss, jaundice and a palpable mass at the right hypochondrium. In some cases a cholangitis may overlap.
Preoperative diagnosis of this disease is made easier by ultrasound and CT. This last technique of investigation is also useful as a guide during the execution of the thin needle aspiration and the biopsy of the lesion.
After the onset of symptoms, spread of the tumor outside the gallbladder by direct extension or lymphatic or bloodstream is almost the rule. Over 75% of gallbladder carcinomas are unresectable at the time of surgery, with the sole exception of tumors discovered by chance during a laparotomy. One-year mortality in the case of unresectable neoplasms is about 95% and only 5% of patients survive for 5 years or longer after diagnosis. Radical surgery does not improve survival and radiotherapy and chemotherapy have also proved to be poorly effective.
cfr Oncology