Week 6 - F - Focal Liver Lesions (2) - Malignant - Hepatocellular, Fibrolamellar and cholangiocarcinoma, metastases Flashcards

1
Q

Overall metastatic disease accounts for >90% of all liver malignancies making the primary liver tumours comparatively rare. What are the two most common primary liver malignancies?

A

Hepatocellular carcinoma accounts for the vast majority of primary liver malignancies Cholangiocarcinoma is the second most common cause of primary liver malignancies

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2
Q

The most important risk factor for hepatocellular carcinoma is cirrhosis What are the different risk factor conditions that may lead to this?

A

Hepaittis B infection - leading cause worldwide * Hepatitis C * Alcohol * NAFLD * Autoimmune hepatitis * Primary biliary cirrhosis * Primary sclerosing cholangitis * Haemachromatosis * Wilson’s disease * A1-antitrypsin deficinecy

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3
Q

What are the common presenting features of hepatocellular carcinoma?

A

HCC presents as An abdominal mass with right upper quadrant pain and weight loss Associated with worsening of pre-existing chronic liver disease or acute liver failure

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4
Q

What is seen on examination in a patient with HCC?

A

On examination * Signs of cirrhosis * Hard enlarged RUQ mass * Liver bruit may occur but are rare

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5
Q

What is the prognosis in a patient with HCC?

A

Prognosis is very poor (overall survival is 15% at 5 years. Usually the tumor is advanced at presentation unless discovered incidentally

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6
Q

What are the routes of metastasis of HCC? Which organs are commonly affected?

A

* Rest of the liver * Portal vein - haematogenous * Lymph nodes * Lung * Bone * Brain

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7
Q

When is screening carried out for HCC and what is involved in the screening?

A

Screening for HCC is carried out in all high risk patients eg patients with cirrhosis or who have chronic HBV infection Alfa-feto protein +/- ultrasound is carried out 6 monthly in these patients

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8
Q

In the diagnosis of HCC, what would the LFTs show? Which protein, normally very low in adults is raised in virtually all cases of HCC? Why should testicles be examined in males when this protein is found to be raised?

A

LFTs * Would show raised ALT and AST * Increased bilirubin and PT * Decreased albumin Alfa-feto protein is raised in virtually all cases of HCC The testis should be examined in males (testicular tumours may cause raised AFP).

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9
Q

How is the diagnosis of hepatocellular carcinoma carried out?

A

Ultrasound scan carried out to screen patients for a liver tumour * Triphasic CT scan - shows very early aterial perfusion or * MRI (usually both) are the imaging modalities of choice

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10
Q

Why are liver biopsies generally avoided?

A

Biopsy should be avoided as it seeds tumours cells through a resection plane.

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11
Q

What is the scoring system used to classify cirrhosis in a patient that can be used to help with choosing a treatment option for the patients liver malignancy?

A

Child-Pugh classification

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12
Q

Patients should be staged with liver MRI and chest, abdomen and pelvic CT scan. When is resection carried out in liver cancer?

A

Resection can be carried out for solitary tumours <3cm across when the patient also has normal portal pressure and bilirubin - usually child-pugh class A Recurrence rate is high

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13
Q

Liver transplantation is the single best available treatment for HCC When may it be considered?

A

Consideration of liver transplantation may be given to patients who * Have a single tumour

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14
Q

For very early disease - resection is usually given if a single small tumour or liver transplantation What is the treatment given early disease - for patients of whom have non resectable cancer? (Barcelona clinic liver cancer staging shown in image)

A

Early disease - treatment usually is with percutaneous RFA (radiofrequnecy ablation)

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15
Q

What is the treatment given for intermediate disease - for patients of whom have non resectable cancer? (Barcelona clinic liver cancer staging shown in image)

A

Intermediate disease - TransArterial ChemoEmbolization - TACE Inject chemotherapy selectively in hepatic artery Then inject an embolic agent Only in patients with early cirrhosis

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16
Q

What is the treatment of advanced HCC (barcelona clinic liver cancer stage C)? How does this drug work?

A

Sorafenib - an oral multikinase inhibitor (of vascular endothelial growth factor receptor, platelet derived growth factor receptor and RAF) First-line targeted treatments for patients with advanced-stage HCC (such as vascular invasion or extrahepatic disease)

17
Q

FIBROLAMELLAR CARCINOMA - very rare What is the typical presentation of this type of liver cancer? Does the patient have primary liver disease?

A

Fibrolamellar carcinoma, or FLC, is a rare cancer of the liver that usually grows in teens and adults under 40 years old. This type of cancer is different than other types of liver cancer because it happens in people who have healthy livers It is not related to cirrhosis and patient has a normal ALP

18
Q

Fibrolamellar carcinoma usually does not have symptoms when the cancer first starts. Later, when the tumor gets larger, symptoms can include what?

A

Can include the same symptoms as HCC * Abdominal pain * Nausea and vomiting * Appetite and weight loss * Malaise (having less energy or just feeling unwell) * Jaundice (yellowing of the skin) * Palpable liver mass Acute liver failure may occur in advanced disease

19
Q

The usual markers for liver disease – aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase – are often normal or only slightly elevated. FHCC often does not produce alpha fetoprotein (AFP), a widely used marker for conventional hepatocellular carcinoma. What is often increased in fibrolamellar carcinoma?

A

It is associated with elevated neurotensin levels - a type of peptide

20
Q

How is fibrolamellar carcinoma diagnosed?

A

Diagnosis is normally made by imaging (ultrasound, CT or MRI) and biopsy CT shows similar to fibronodular hyperplasia - typical stellate scar with radial septa showing persisting enhancement (makes it resemble fibronodular hyperplasia)

21
Q

What is the treatment of fibrolamellar carcinoma?

A

Surgical resection or transplantation is the standard of care for fibrolamellar carcinoma TACE for patients with unresectable tumour

22
Q

CHOLANGIOCARCINOMA Thee second most common type of primary liver malignancy is cholangiocarcinoma What does cholangiocarcinoma arise from? Where in the hepatobiliary system do they tend to arise?

A

Cholangiocarcinoma is carcinoma of the bile ducts (cholangiocytes are the cells lining the bile ducts) - the majority of tumours being extrahepatic Majority that arise are peri hilar (confluence of right and left hepatic ducts - so extra hepatic)

23
Q

What are the risk factors for cholagniocarcinoma?

A

The major risk factor for this disease is a patient with primary sclerosing cholangitis Other risk factors include ulcerative colitis, hepatitis B and C

24
Q

How does cholangiocarcinoma present? How do LFTs look?

A

Patient typucally presents at late stage with Fever, adbo pain +/- ascites , malaise, jaundice An obstructive jaundice picture - raised ALP and GGT, and bilirubin

25
Q

Which markers are often raised in the blood for cholangiocarcinoma?

A

Serum levels of carcinoembryonic antigen (CEA), CA-125 and CA19-9 are often elevated, but are not sensitive or specific enough to be used as a general screening tool.

26
Q

How is cholangiocarcinoma diagnosed?

A

US is the initial investigation usually carried out CT/ MRI and MRCP/ERCP are the diagnostic imaging methods of choice.

27
Q

The majority of tumours are perihilar for cholangiocarcinoma What is the 4 different gradings for these tumours(modified Bismuth-colette classfication for hilar cholangiocarcinoma)

A

Type 1 - below the confluence Type 2 - confined to the confluence Type 3a - extension into the right hepatic duct Type 3b - extension into the left hepatic duct Type 4 - extension into the right and left hepatic duct

28
Q

What is the treatment options for patients with cholangiocarcinoma? (for resectable and non-resectable tumours)

A

If resectable Surgical resection – Only chance of cure Bile duct and liver resection If not resectable Pallation - biliary stent due to obstruction caused by the tumour (ECRP insertion)

29
Q

Secondary liver metastases are the most common site for blood born metastases What are the common primaries that metastasize here?

A

Common primaries - breast, pancreas, stomach, colon, lung

30
Q

How is treatment of a secondary liver metastases usually treated?

A

Treatment and prognosis vary with type and extent of primary tumour - in some cases resection or chemoembolisation (TACE - transarterial chemoemblisation) may be possible