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Liver and Biliary Tract Tumors

Notes

Liver and Biliary Tract Tumors

Sections





Liver & Biliary Tumors

Benign:

  • Hepatic hemangiomas
  • Focal nodular hyperplasia
  • Hepatic adenoma

Malignant:

  • Hepatocellular carcinoma
  • Hepatic angiosarcoma
  • Cholangiocarcinoma

Common Metastases include the breast, lung, and gastrointestinal tract.

Signs/Symptoms:

Liver and biliary tumors are usually asymptomatic, until they become advanced or too large.

Advanced/large: patients may report right upper quadrant discomfort, nausea, bloating, and signs or symptoms of liver disfunction.

Treatment:
Includes resection or transplant; chemotherapy or other treatments may also be necessary.

Benign Tumors

Hepatic hemangiomas

These are most common benign liver tumors; that they are mesoderm-derived lesions that comprise malformed blood vessels.

"Cavernous" is the most common subtype, so be aware that these are sometimes called "cavernous hemangiomas."

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See image references below.

They can be found as solitary or multiple lesions, and range in size from 1 mm to 50 cm.

Tumors smaller than 5 cm are typically asymptomatic; however, larger tumors can cause discomfort, nausea, and bloating.

Kasabach-Merrit syndrome: "Giant" hemangiomas, those that are 5 cm or greater, are associated with Kasbach-Merrit syndrome.

This is a life-threatening disorder marked by: Hemangioma, Thrombocytopenia (low platelet count, and disseminated intravascular coagulation (abnormal clotting in vessels depletes clotting factors and causes excessive bleeding).

KMS is most often due to congenital defects and found in infants.

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See image references below.

Focal nodular hyperplasia

Comprises densely packed hepatocytes fed by an enlarged artery and surrounded by fibrous tissue (note that the nodules are similar to cirrhotic nodules).

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See image references below.

These solid tumors are caused by vascular abnormalities; hyper- or hypo-perfusion can induce a regenerative response leading to hyperplasia.

Lesions are usually solitary, and range 4-8 cm in size.

They are more common in women aged 20-50.

Hepatic adenomas
Aka, hepatocellular adenomas

These are rare epithelial tumors (there are three molecular subtypes); they are usually solitary, light brown or yellow in color, and soft without a capsule, and are more commonly found in the right lobe.

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See image references below.

These tumors are associated with elevated estrogens (as in oral contraceptive users), anabolic androgens, glycogen storage diseases, and metabolic syndrome.

Although usually asymptomatic, these tumors can rupture and cause acute hemoperitoneum, with severe abdominal pain, hypotension, and shock. Treat this with transarterial embolization and, if needed, resection.

Malignant Tumors

Hepatocellular cancer

Note fibrosis (blue), Mallory-Denk bodies, and atypical nuclei.
See references below.

Hepatocellular cancer is in the top ten most common cancers worldwide.

Associated with chronic liver disease, and specifically cirrhosis.

Historically, Hepatitis B and C infections were the most common precursors, but, in western countries, fatty liver disease is a growing cause.

Thus, in patients with chronic liver disease, we do routine surveillance via ultrasound to look for signs of hepatocellular carcinoma – early diagnosis leads to better prognosis:

Early stage 5-year survival rate is about 35%, whereas late stage with metastasis has a 5-year survival rate of only 3%.

Radiological hallmark: "Wash-in"/"wash-out" with dynamic CT or MRI: during the arterial phase, we see the "wash-in" contrast effect; in the venous phase, we see the "wash-out" effect.

Hepatic angiosarcomas

These are rare but aggressive mesenchymal tumors that metastasize to the lungs, spleen, and bone with a high recurrence rate and poor prognosis.

They comprise enlarged anastomosing vascular channels with overgrown endothelial cells, and are known to cause spontaneous hemoperitoneum.

They are more common in males, and are associated with exposure to environmental toxins, such as vinyl chloride, arsenic, radiation, etc.

Note hemorrhaging and necrosis.
See image references below.

Cholangiocarcinomas

Biliary tract tumors that are named for their location:

  • Intrahepatic cholangiocarcinomas are located proximal to the second-order bile ducts.
  • Perihilar cholangiocarcinomas are located between the second-order bile ducts and the opening of the cystic duct.
  • Distal cholangiocarcinomas are in the common bile duct distal to the cystic duct opening.

These tumor types have their own unique features, but all are the result of chronic bile duct inflammation that transforms the cholangiocytes.

Common causes of cholangiocarcinoma vary by location:

  • In Western countries, many cases are caused by primary sclerosing cholangitis.
  • In Southeast Asia, many cases are caused by flatworm infection.

As we might expect, later stages of cholangiocarcinoma are characterized by jaundice due to biliary tract obstruction.

Board Review

Liver & Biliary Tract Tumors

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References

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    ResearchGate. "Fig. 4. Hepatic Hemangioma. A, Medium Magnification Showing Vascular..." Accessed July 14, 2022. https://www.researchgate.net/figure/Hepatic-hemangioma-A-Medium-magnification-showing-vascular-channels-lined-by-bland-and_fig2_282313555.
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  • Mansour, Subhi, Sharbel Khouri, Maisa Andraous, and Safi Khuri. "Extremely Large Hemangioma of the Liver: Safety of the Expectant Management." Archives of Surgery and Clinical Research 3, no. 2 (September 6, 2019): 061–064. https://doi.org/10.29328/journal.ascr.1001036.
  • Myers, Lauren, and Joseph Ahn. "Focal Nodular Hyperplasia and Hepatic Adenoma." Clinics in Liver Disease 24, no. 3 (August 2020): 389–403. https://doi.org/10.1016/j.cld.2020.04.013.
  • Reig, Maria, Alejandro Forner, Jordi Rimola, Joana Ferrer-Fàbrega, Marta Burrel, Ángeles Garcia-Criado, Robin K. Kelley, et al. "BCLC Strategy for Prognosis Prediction and Treatment Recommendation: The 2022 Update." Journal of Hepatology 76, no. 3 (March 1, 2022): 681–93. https://doi.org/10.1016/j.jhep.2021.11.018.
  • Rujeerapaiboon, Natthapat, and Panu Wetwittayakhlang. "Primary Hepatic Angiosarcoma: A Rare Liver Malignancy – Varying Manifestations but Grave Prognosis." Case Reports in Gastroenterology 14, no. 1 (2020): 137–49. https://doi.org/10.1159/000506928.
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Images:

  • Hepatic hemangioma: https://commons.wikimedia.org/wiki/File:Cavernous_liver_hemangioma_-_high_mag.jpg
  • KMS: https://commons.wikimedia.org/wiki/File:Meritt_kasabach_syndrome.jpg
  • Focal nodular hyperplasia: https://commons.wikimedia.org/wiki/File:Focal_nodular_hyperplasia_-high_mag.jpg#/media/File:Focal_nodular_hyperplasia-_intermed_mag.jpg
  • Hepatic adenoma: https://www.flickr.com/photos/radquiz/4276539861/
  • Hepatocellular carcinoma: https://commons.wikimedia.org/wiki/File:Hepatocellular_carcinoma_low_mag.jpg
  • Hepatic angiosarcoma: https://en.wikipedia.org/wiki/Liver_angiosarcoma#/media/File:Gross_specimen_of_liver_angiosarcoma.jpg