Surgical Diseases of the Liver and Bile Ducts
Transplant surgeons at BIDMC provide surgical expertise for a number of different hepatobiliary conditions brought on by liver infection, liver cysts, benign liver masses, primary (original) and metastatic (spreading) hepatic malignancies, and portal hypertension. Some of the more common hepatobiliary diseases and conditions surgeons and physicians in the Transplant Institute treat include the following:
Infections involving the liver are primarily bacterial abscesses (pyogenic or pus-producing), parasitic abscesses (amebic or echinococcal, two types of parasites) or viral (hepatitis A, B, C, D and E). An abscess is a collection of pus within a confined space or organ. Treatment for liver infections can include antibiotics with or without percutaneous (through the skin) or surgical drainage procedures. However, cysts associated with hydatid disease, a type of liver infection, must always be surgically removed.
Hepatitis can result from viral infection of the liver cells, causing inflammation, swelling, cell death and ultimately lead to liver destruction or scarring. There are many different types of viral hepatitis - A, B, C, D, and E - that specifically infect the liver. Some other viral infections that affect the entire body can also cause liver inflammation, such as herpes infection, cytomegalovirus, and Epstein Barr Virus, as part of their disease process. Hepatitis C virus (HCV) is the leading cause of liver transplants in the United States. Hepatitis B (and D) also cause chronic liver disease. Hepatitis B (HBV) is the leading cause of liver cancer worldwide.
Cysts can be divided into benign (non-cancerous) congenital cysts (present at birth) or acquired cysts; that is, a new cyst caused from a traumatic injury, for instance. Cysts present at birth may result from abnormally developed bile ducts. Physicians use the term solitary cyst to refer to liver cysts that are not associated with the inherited polycystic liver disease, even though solitary cysts may be multiple in nature. Polycystic (having many cysts) liver disease can affect the liver, kidneys, lung and pancreas. Additionally, some patients are prone to neoplastic cysts, where the liver tissue grows abnormally, forming a mass that can either be benign (non-cancerous) or malignant (cancerous).
Sometimes surgeons can treat congenital or acquired cysts by aspirating, or using suction, to remove the fluid. In other cases it is necessary to surgically remove them and this can usually be done laparoscopically.
Benign Liver Masses
Many disease areas in the liver may appear as single or multiple liver masses that are benign (non-cancerous). The most typical benign liver masses include cavernous hemangioma, hepatocellular adenomas and focal nodular hyperplasia.
Cavernous hemangioma is one of the more common liver tumors. Tangled and multiple blood vessels, supported by walls of collagen (the fibrous protein found in cartilage, bone and tendon), characterize this benign tumor, generally found more in women than men. It is very rare that a hemangioma will burst and they never become cancerous. If the hemangioma is large and causing discomfort, then it is surgically removed.
Hepatocellular adenomas are non-cancerous liver tumors typically found in women who use birth control pills. Adenomas can also occur in men who have a history of steroid use. Because of the risk of bleeding, rupture and the small chance that the tissue could turn cancerous, surgery is the treatment of choice for most large adenomas in patients who are suitable surgical candidates. Small adenomas may be observed and monitored in some cases.
Focal nodular hyperplasia (FNH) is a highly common, benign tumor of the liver. The risk for bleeding or the development of cancer has not been documented. Treatment depends on the certainty of the diagnosis, as the most difficult challenge is to distinguish FNH from cancerous liver tumors or adenomas. If it is not possible to definitively rule out a cancer, then surgery is the safer choice.
Primary Malignancies of the Liver, Gallbladder and Biliary Tree
Primary (meaning they start in the liver) malignant (cancerous) tumors of the liver include hepatocellular carcinoma, cholangiocarcinoma, hepatoblastoma, and a number of other less frequently occurring cancerous tumors.
Hepatocellular carcinoma (HCC) accounts for the overwhelming majority of primary liver cancer and Hepatitis B (HBV) is the leading cause of HCC worldwide. Hepatitis C (HCV) is also a common cause of HCC in the United States, but all patients with cirrhosis of the liver are at increased risk for developing HCC. Surgical resection to remove the tumor is the best treatment depending on the extent of disease. Survival rates are better when tumors are small and can be completely removed. Patients who have severe liver disease and portal hypertension may not be candidates for surgical resection, but may be best treated by liver transplantation. However, when tumors invade both lobes of the liver (multifocal), have spread outside the liver (for example, to the lungs), or in cases where the tumor in the liver originated from some other site in the body and then spread to the liver, surgery is generally not recommended. In this case, other treatment options include:
- Chemotherapy - either systemic (treating the entire system) or intra-arterial (delivering the chemotherapy through a catheter directly into the artery supplying the liver)
- Radiofrequency ablation (placing a probe into the tumor and heating up the tumor using radio waves in order to shrink/destroy the cancerous tissue)
- Chemoembolization (injecting small particles containing a chemotherapy drug into the hepatic artery to block blood supply to the tumor and shrink/destroy the cancerous tissue)
- Radiation therapy (targeting the tumor with X-rays or radioactive agents)
- Cyberknife® (precisely targeted radiation therapy, beamed at the tumor site, with minimal exposure to surrounding healthy tissue); Cyberknife® is especially well suited for hard-to-reach and complex tumors in the liver and pancreas, among other organs.
Physicians continue to evaluate newer non-surgical approaches to treat HCC.
Normally the liver can regenerate (grow back) after a portion has been removed, but this ability is limited if you have cirrhosis (or scarring) of the liver. As mentioned above, if you also have extensive HCC that cannot be removed surgically, or that does not respond to other treatment procedures, you may be a candidate for liver transplantation.
Cholangiocarcinoma is cancer of the bile duct system (the biliary tree). This can arise in the liver from the bile duct cells or in the major duct draining bile from the liver called the common bile duct. A slow growing, rare type of cancer that often occurs at the junction of the right and left hepatic ducts as they leave the liver (Klatskin's tumor), surgical resection is the treatment of choice. If the tumor cannot be removed surgically, liver transplantation may be an option for some selected patients, and for other patients treatment includes chemotherapy and radiation.
Gallbladder carcinoma is an uncommon cancer that may arise anywhere in the gallbladder and frequently invades into the adjacent liver tissues. Surgical treatment offers the best hope for cure if the tumor has not spread elsewhere and can be resected. This includes removing the gallbladder, the surrounding liver tissue, and the lymph nodes around the liver. Sometimes the common bile duct must be removed to achieve clear surgical margins.
Hepatoblastoma is a highly malignant tumor that occurs almost exclusively in children less than five years of age, although older children and adults have been diagnosed with this condition as well. A large, single tumor that is usually found in the right lobe of the liver, hepatoblastomas are generally associated with abnormalities at birth. Surgery to remove the tumor is the preferred treatment. Radiation and/or chemotherapy before surgery may benefit some patients. If the tumor is not surgically removable, liver transplantation may be an option.
Metastatic Malignancies of the Liver
Metastases are cancer cells that have spread from an original or primary site to one or more locations or organs elsewhere in the body. For example, cancer that begins in the colon (primary) can metastasize to the liver. The liver is a prime site for metastatic cancer for many reasons; chiefly, the liver receives 80 percent of its blood supply from the portal vein that receives the blood draining from the many primary cancer sites, such as the colon and rectum.
The most common primary tumors that cause liver metastases include lung, colon, pancreas, breast, stomach, ovary, prostate, gallbladder and cervix. If there is not evidence of other systemic disease or if the systemic disease is well-controlled, surgical resection is the treatment of choice if all the tumor can be removed and still leave an adequate amount of liver tissue. If surgery is not an option then chemotherapy, radiofrequency ablation, chemoembolization, or Cyberknife® may be used.
Portal hypertension is a common complication of cirrhosis, which in turn may be caused by many different liver diseases in which normal, healthy tissue is replaced with nonfunctioning "scar" tissue, and healthy, functioning liver cells are lost. With portal hypertension, scar tissue prevents the vein that drains blood from the spleen and intestines (portal vein) from bringing the blood through the liver. This condition creates increased pressure in the veins, which can lead to other problems, such as fluid buildup (ascites) in the abdomen and elsewhere, and enlarged veins (varices) in the esophagus or stomach that can bleed. Treatment options to manage complications from portal hypertension include the following.
Endoscopic treatment in the Center for Interventional Gastroenterology: Gastroenterologists (physicians who treat disorders of the digestive tract) use a lighted, flexible tube (introduced through the mouth) to visualize, diagnose and stop bleeding varices by using rubber bands to block the vessels ("banding") or by injecting a solution into the varices so they scar ("sclerotherapy").
Medication: Physicians may prescribe medication (separately or together with endoscopic therapy) to lower variceal pressure and reduce the likelihood of bleeding.
Diet: It is important to follow a low sodium diet (typically 2 grams/day) to avoid fluid collection (ascites) and minimize edema (swelling). Our nutritionist can explain which foods are high in sodium, what alternatives and substitutes you might consider, and how to interpret food labels. Also, to help prevent encephalopathy (confusion, drowsiness and behavioral changes brought on by liver disease), you must take your medications exactly as directed. Protein is not restricted in the case of encephalopathy; however, the type of protein you eat is important. For example, our nutritionist may advise you to eat a mix of protein foods from meat and vegetable sources such as eggs, dairy products, nuts, lentils and beans, but to consume less red meat. Also, you may be able to better tolerate protein-rich foods if you eat them throughout the day rather than all at once. Click
here to read more about diet and nutrition.
Specialized Portal Decompressive Surgery: If physicians cannot control bleeding varices with endoscopic therapy or medication, then surgeons at the Transplant Institute perform highly specialized portal decompressive surgery to reconfigure blood flow through the liver. Portal decompressive surgery, which can include portacaval or other shunting procedures, reduces pressure in the veins draining the liver, and in so doing, decreases the swelling in the variceal veins to stop the bleeding and lessen the risk of rupture. These procedures are done by sewing a vein such as the portal vein or splenic vein, which are under high pressure, to a vein under low pressure.
TIPS, or Transjugular Intrahepatic Portosystemic Shunt, is a specialized procedure used to treat portal hypertension. Radiologists insert a metal stent in the liver, via the jugular vein in the neck. The stent (a tiny wire mesh tube), creates a passageway between the portal and hepatic veins to promote blood flow through the liver from the portal vein directly to the hepatic vein thereby bypassing the blockage caused by the scarring of cirrhosis and relieving the high pressure in the liver. However, some patients may have vein abnormalities (such as a clot in the portal vein) that prohibit the TIPS procedure or may be at greater risk for mental confusion (encephalopathy) following the procedure.
The Budd-Chiari Syndrome, an uncommon syndrome characterized by obstruction of veins draining blood out of the liver, can also cause portal hypertension. Some patients are born with a membrane-type web that actually blocks blood flow. Patients experience abdominal pain, ascites (fluid collection in the abdomen) and abnormal liver function. A radiology exam confirms the diagnosis by highlighting blockage in the hepatic veins. Most patients need surgery to improve, either to repair the membranous webs or to perform a side-to-side portacaval shunt procedure (sewing the portal vein to the inferior vena cava) to reduce the high blood pressure in the liver.