Pathology of Liver Tumors Flashcards

1
Q

What is a Von Meyenburg Complex and what causes it?

A

Bile duct hamartoma - caused by persistence of embryonic bile duct structores

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2
Q

What does a Von Meyenburg Complex look like and why is it important to identify it?

A

Looks like irregular, dilated bile ducts within fibrous connective tissue, and there are frequently multiple

Must be differentiated from adenocarcinoma with desmoplasia. Note: these will have non cellular atypia

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3
Q

What is the pathogenesis of nodular hyperplasias?

A

Alterations in blood supply to the liver (decreased portal vein blood flow, increasing hepatic artery blood flow)

-> too much well oxygenated blood induces growth factors which produce a nodular hyperplasia of hepatocytes which can be seen discretely on imaging

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4
Q

What does a focal nodular hyperplasia look like grossly?

A

Solitary, well-circumscribed, pale, firm lesion (<5 cm), with central stellate scar surrounded by parenchymal nodules between fibrous septae radiating outwards.

Looks alot like oncocytoma of kidney

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5
Q

Who tends to get focal nodular hyperplasia and how is it found?

A

Occurs in young adults, especially with oral contraceptives in women, or anabolic steroids in men

Usually found as an incidental finding

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6
Q

What are the microscopic features of focal nodular hyperplasia, and what are the clinical consequences?

A

Central scar, with abnormally large arterial branches accompanied by bile ductular proliferation (which don’t drain anything) and chronic inflammation, with no well defined portal triads

Hyperplasia grows between radial spokes of fibrosis

There are no clinical sequellae -> just need to differentiate between important masses

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7
Q

What are the gross and microscopic features of nodular regenerative hyperplasia? What is it difficult to differentiate from?

A

Gross - diffuse, pale, fine nodularity of the liver

Microscopic - Regenerative nodules WITHOUT fibrosis (check trichrome stain), often with obliterated portal vein branches

Lack of fibrosis will help differentiate from cirrhosis

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8
Q

Who is at risk for nodular regenerative hyperplasia and what is the clinical consequence?

A

Those with a predisposing underlying condition leading to reduced blood flow to liver: especially hematologic / neoplastic disorders (decrease blood flow due to thrombosis)

Clinical consequence: Portal hypertension can develop, like cirrhosis

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9
Q

What is the most common benign hepatic tumor? How does it appear grossly?

A

Cavernous hemangioma - can be detected on imaging

Appears grossly as a subcapsular, small, well-demarcated, soft, red-blue lesion

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10
Q

How does cavernous hemangioma appear microscopically?

A

Large, thin-walled, blood-filled spaces lined by normal-appearing endothelial cells, separated by scarce fibrous connective tissue

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11
Q

How should cavernous hemangioma be treated?

A

Do not biopsy - will bleed

Just need to differentiate from more ominous lesions

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12
Q

What benign liver neoplasm is most associated with oral contraceptive or estrogen use, and almost never arises outside of this context? How does it present clinically?

A

Hepatocellular adenoma

Present in young women on oral contraceptives. May cause an acute abdomen due to rupture and intraperitoneal hemorrhage

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13
Q

What do you need to differentiate hepatocellular adenoma from? Is it totally benign?

A

Need to differentiate from hepatocellular carcinoma

Not totally benign - may transform into hepatocellular carcinoma if Beta-catenin mutations are present

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14
Q

How does hepatocellular adenoma appear grossly? What is a common complication?

A

Solitary, well-circumscribed mass, which is tan-green (benign hepatocytes make bile), and is often subcapsular

Strangely, it is more likely to bleed that the cavernous hemangioma (another benign tumor located at the same spot made of blood vessels)

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15
Q

How does hepatocellular adenoma appear microscopically?

A

Sheets and cords of relatively normal-looking hepatocytes, containing glycogen or triglycerides (functioning properly) but with NO portal tracts and scattered arteries and veins which are prone to hemorrhage.

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16
Q

What is the most common liver malignancy and how does it tend to arise?

A

Metastasis to liver

Colon, pancreas, lung (spreads via systemic circulation thru hepatic artery), and breast (after lung metastasis)

17
Q

What are the early vs late lab elevations in metastasis to liver?

A

Early - elevated Alk Phos (indicates metastasis to liver) and LDH (nonspecific for cell death)

Late - increased serum bilirubin and transaminases - due to necrosis of hepatocytes from ischemia and blockage of bile drainage = HEPATOMEGALY

18
Q

What is the most common primary liver malignancy? What is it also called on the wards? Where in the world is its highest incidence?

A

Hepatocellular carcinoma

Called “Hepatoma” :(

Incidence reflects prevalence of HBV infection, especially southeast Asia

19
Q

What are the three major risk factors for development of HCC? Which is most common in US?

A
  1. Asymptomatic HBV carrier state
  2. Cirrhosis (repeated cycles of necrosis and regeneration is progressively mutagenic) - most common in US
  3. Exposure to aflatoxin
20
Q

How does HBV predispose to HCC? What is the best way to break this cycle?

A

HBV DNA integrates into hepatocellular genome, activation of oncogenes and production of HBV X-protein (transcriptional activator of multiple pro-proliferation and anti-repair genes)

Best way to break cycle: vaccination to prevent vertical transmission

21
Q

What is aflatoxin and why is it carcinogenic?

A

Toxin produced by Aspergillus flavus, which can contaminate stored peanuts and grains -> think of the cow next to the tractor in sketchy

Carcinogenic because it induces p53 mutations

22
Q

What things are associated with the development of cirrhosis which predisposes to HCC?

A

HCV, HBV, alcohol, nonalcoholic fatty liver disease, hemochromatosis, Wilson disease, and A1AT deficiency

23
Q

How does hepatocellular carcinoma appear grossly?

A

Solitary, multinodular, or diffuse tan-green mass in regions of hemorrhage or necrosis

-> often has associated venous invasion (invades like a snake like renal cell carcinoma)

24
Q

What are the clinical consequences of venous invasion by HCC?

A

Portal vein -> portal hypertension

Hepatic vein -> Budd-Chiari syndrome, can even extend to right atrium thru IVC

25
Q

What is the clinical triad of Budd-Chiari syndrome? What is seen in the liver?

A

Sudden liver enlargement (hepatomegaly), pain, and ascites - due to acute hepatic venous occlusion

Centrilobular congestion and necrosis can be seen

26
Q

How does HCC appear microscopically?

A

highly variable. May be trabeculated or pseudoacinar if more well differentiated.

Can be told apart from benign by presence of prominent nucleoli, eosinophilic cytoplasm, and lack of simple hepatocellular cords (tend to grow in clusters / acini)

27
Q

What is one marker which is associated with hepatocellular carcinoma?

A

Elevated serum alpha-fetoprotein

-> malignant hepatocytes express the fetal form of albumin

28
Q

What is the prognosis of hepatocellular carcinoma and why?

A

Poor -> usually spreads hematogenously due to lungs by the time there is a presenting complaint (i.e. Budd-Chiari syndrome) -> coagulopathy already spreading outside of liver

May be detected early by frequent ultrasounds in cirrhotic individuals

29
Q

What form of hepatocellular carcinoma is seen in young adults and has a good prognosis? Why is the prognosis good?

A

Hepatocellular carcinoma, fibrolamellar variant

Prognosis is good because it is well circumscribed and individuals often have no underlying liver disease (can resect just fine)

30
Q

How does the fibrolamellar variant of hepatocellular carcinoma appear grossly and microscopically?

A

Grossly - alot like focal nodular hyperplasia or oncocytoma -> firm, white to green mass in otherwise normal appearing liver

Microscopically - bands of well-differentiated tumor cells but with prominant nucleoli coursing through abundant fibrous stroma with parallel lamellae of collagen

31
Q

What is cholangiocarcinoma? What are the risk factors?

A

Carcinoma of the bile duct epithelium (intrahepatic or extrahepatic)

Risk factors: often absent, but include primary sclerosing cholangitis, congenital biliary tract abnormaliites leading to stasis, Thorotrast exposure, and Clonorchis sinesis (liver fluke)

32
Q

How does cholangiocarcinoma appear grossly and microscopically?

A

Grossly - pale, hard mass

Microscopically - moderately differentiated adenocarcinoma surrounding by sclerotic desmoplasia (hard to differentiate from other adenocarcinomas)

33
Q

What is the prognosis of cholangiocarcinoma and which is the worst?

A

Very poor -> frequent metastases and is detected late

Intrahepatic is worst type -> detected very late (other ducts can drain bile)

34
Q

What type of liver cancer is PVC (vinyl chloride) associated with and how does it appear grossly and microscopically? Whats the prognosis?

A

Angiosarcoma (think malignant hemangioma)

Gross - Multiple hemorrhagic nodules and blood-filled cysts

Microscopic - Atypical endothelial cells and irregular vascular spaces

Shit prognosis

35
Q

What tumor is associated with an elevated alpha-fetoprotein in children? What’s the prognosis?

A

Hepatoblastoma

Aggressive malignancy, but good prognosis with surgical resection and chemotherapy

36
Q

How does hepatoblastoma appear grossly and microscopically?

A

Grossly - Large, hemorrhagic, necrotic nodular mass

Microscopically - small, round, blue-cell tumor with evidence of epithelial (embryonal to fetal hepatocellular) differentiation +/- mesenchymal differentiation (may even have random bone deposits)