18.8

Question 1

what is focal nodular hyperplasia (FNH)

Answer 1

benign

usually an incidental finding on young-middle age adults

highly characteristic for single well demarcated lesions with central scar & “maplike pattern” of cytoplasmic GS staining

Question 2

when can you get multifocal FNH

Answer 2

in association w/ hepatic/extrahepatic hemangioma, vascular malformations and some brain tumors

Question 3

what is nodular regenerative hyperplasia (NRH)

Answer 3

benign, however it can impede blood flow;

multiple nodules but no fibrosis (differentiate from cirrhosis this way.)

Pts may develop portal HTN.

associated with HIV and rheumatologic dz’s

Question 4

what are cavernous hemangiomas

Answer 4

MC benign neoplasm of the liver

females and usually an incidental findings

It can however can be a life threatening intraabd emergency from hemorrhage due to subscapular locations

mistake for malignancy

Question 5

what is hepatocellular adenoma

Answer 5

benign neoplasms that are more common in young women

strong association with use of OC and anabolic steroids

increase in incidence, possibly due to obesity and metabolic syndromes

incidental finding during imaging, however it can cause pain from rapid growth/hemorrhage/rupture

Question 6

what are the three subtypes of hepatocellular adenoma

Answer 6

HNF1-a inactivated adenoma

inflam adenoma

B-catenin activated adenoma

Question 7

what is the presentation and characteristics of HNF1-a inactivated adenoma

Answer 7

40-50% hepatocellular adenoma cases,

Female

fat w/o atypia

minimal risk for HCC, OC association

Question 8

what is the presentation and characteristics of inflam adenoma

Answer 8

40-50% of hepatocellular adenomas

Female, obesity/metabolic syndrome;

10% also have B-catenin activating mutation -

higher risk of HCC

mimic FNH; increase CRP and amyloid A

Question 9

what is the presentation and characteristics of B-catenin activated adenoma

Answer 9

MEN (40%) & women also,

high risk for HCC,

OC and anabolic steroids association

Question 10

how does hepatoblastoma present

what is it associated w/

Answer 10

MC liver tumor of early childhood (<3 y/o)

abd swelling in asymp infant/child

The baby can also have jaundice or pruritus bc of liver dysfxn

associated w/ FAP and Beckwith-Wiedemann Syndrome

Question 11

what is the prognosis of hepatoblastoma

Answer 11

20% metastasize to the lungs by the time of Dx

It is very fatal w/o treatment

w/ therapy the 5 yr survival rate is 80%

Question 12

what are the two types of hepatoblastoma

Answer 12

1. Epithelial type- small polygonal fetal cells or smaller embryonal cells forming acini, tubules or papillary structures vaguely recapitulating liver development
2. Mixed epithelial/mesenchymal type- mesenchymal differentiation that may consist of primitive mesenchyme, osteoid, cartilage or striated M

Question 13

what is the prevalence of HCC

Answer 13

(MCC primary epithelial liver tumors)

5.4% all CA w/w, with >85% in Asia (SE China, Korea, Taiwan) and sub-saharan Africa (aka places where chronic HBV is commonly transmitted vertically)

incidence has increased in the western world because of HCV and metabolic syndrome.

> men

Question 14

what may be the underlying factor of HCC

Answer 14

HBV/HCV & toxins (aflatoxin & alcohol (MC))

a1-AT, hemochromatosis,

NAFLD/metabolic syndrome,

wilson dz

and precursor lesions

Question 15

what is aflatoxin

Answer 15

Aflatoxin is found in warm humid places w/w and has high risk of HCC.

comes from the Aspergillus flavus/parasiticus family - found in corn, peanuts, cotton seeds and tree nuts.

Question 16

Most HCC present w/…

Answer 16

CLD w/ cirrhosis

Question 17

how do you treat HCC

Answer 17

cirrhotic and non-cirrhotic livers w/ adequate fxn is surgical resection

advanced cirrhosis, liver transplants

does not reach criteria for transplant & cannot be resected then a tumor ablation w/ alcohol or radiofrequency

Question 18

what is the outcome of HCC

Answer 18

metastasis, esp to lungs, is possible late in the dz

LN metastasis is rare

The outcome of HCC is poor bc of the liver dz and HCC resistance to chemo (30% 5 yr survival and only 5-10% if extrahepatic spread)

Question 19

what is the presentation and characterisitcs of fibrolamellar carcinoma

histology?

prognosis?

Answer 19

85% are <35 yo

= single, large, “scirrhous” tumor w/ fibrous bands

Histo: large polygonal cells w/ granular (oncocytic) cytoplasm due to abundant mitochondria; vesicular nuclei w/ prominent nucleolus and parallel lamellae of dense collagen bundles.

Outcomes for the variant (fibrolamellar) are better (40% 10 yr survival)

Question 20

what is cholangiocarcinoma

what is the prevalence

Answer 20

Intrahepatic cholangiocarcinomas are 2nd MCC primary epithelial liver tumors (1st=HCC)

=adenocarcinomas of the intrahepatic biliary tree

increase in incidence in the USA and are VERY common in SE Asia (liver fluke endemic areas - Thailand, Laos, Cambodia)

Question 21

what is the RF for intrahepatic cholangiocarcinomas

Answer 21

Chronic liver dz (HBV, HCV, NAFLD)

fibropolycystic liver dz

chronic inflam : PSC, liver fluke (opisthorchis/chonorchis)

hepatolithaisis

Question 22

what are tumors from the extrahepatic bile duct called

what are RF for these

Answer 22

biliary adenocarcinomas (type of cholangiocarinoma)

RF- developmental disorders (fibropolycystic liver dz), chronic inflam involving the bile duct (PSC, liver flukes- Opisthorchis/clonorchis) and hepatolithiasis

Question 23

what do intra/extrahepatic cholangiocarcinomas look like microscopically

Answer 23

features like adenoCA

mod differentiation and clearly defined glandular/tubular structures lined by malignant epithelial cells in abundant fibrous stroma

Question 24

how do cholangiocarcinomas present

what is the prognosis

Answer 24

Intrahepatic - incidentally or may present w/ cholestasis/symptomatic mass

Extrahepatic - presents w/ biliary obstruction signs.

Lymphovascular and perineural invasion is common

Recurrence is common and prognosis is poor.

Question 25

what are the associatons fo angiocarcinoma

Answer 25

rare

association w/ vinyl chloride, arsenic and thorotrast

malignancy is rare

Question 26

what are characterisitics fo hepatic lymphoma

Answer 26

rare;

MC = diffuse B cell lymphoma at extranodal sites; followe by MALToma

hepatosplenic T cell lymphoma (mid-young adult male - growth w/i spleen, liver, and bone marrow)

rarely associated w/ HBV, HCV, HIV and PBC

Question 27

what are common sites of hepatic metastasis

Answer 27

colon, breast, lungs and pancreas

(metastasis to the liver is more common than primary liver neoplasia)