18.8 Flashcards
what is focal nodular hyperplasia (FNH)
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
when can you get multifocal FNH
in association w/ hepatic/extrahepatic hemangioma, vascular malformations and some brain tumors
what is nodular regenerative hyperplasia (NRH)
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
what are cavernous hemangiomas
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
what is hepatocellular adenoma
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
what are the three subtypes of hepatocellular adenoma
HNF1-a inactivated adenoma
inflam adenoma
B-catenin activated adenoma
what is the presentation and characteristics of HNF1-a inactivated adenoma
40-50% hepatocellular adenoma cases,
Female
fat w/o atypia
minimal risk for HCC, OC association
what is the presentation and characteristics of inflam adenoma
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
what is the presentation and characteristics of B-catenin activated adenoma
MEN (40%) & women also,
high risk for HCC,
OC and anabolic steroids association
how does hepatoblastoma present
what is it associated w/
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
what is the prognosis of hepatoblastoma
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%
what are the two types of hepatoblastoma
- Epithelial type- small polygonal fetal cells or smaller embryonal cells forming acini, tubules or papillary structures vaguely recapitulating liver development
- Mixed epithelial/mesenchymal type- mesenchymal differentiation that may consist of primitive mesenchyme, osteoid, cartilage or striated M
what is the prevalence of HCC
(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
what may be the underlying factor of HCC
HBV/HCV & toxins (aflatoxin & alcohol (MC))
a1-AT, hemochromatosis,
NAFLD/metabolic syndrome,
wilson dz
and precursor lesions
what is aflatoxin
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.
Most HCC present w/…
CLD w/ cirrhosis
how do you treat HCC
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
what is the outcome of HCC
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)
what is the presentation and characterisitcs of fibrolamellar carcinoma
histology?
prognosis?
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)
what is cholangiocarcinoma
what is the prevalence
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)
what is the RF for intrahepatic cholangiocarcinomas
Chronic liver dz (HBV, HCV, NAFLD)
fibropolycystic liver dz
chronic inflam : PSC, liver fluke (opisthorchis/chonorchis)
hepatolithaisis
what are tumors from the extrahepatic bile duct called
what are RF for these
biliary adenocarcinomas (type of cholangiocarinoma)
RF- developmental disorders (fibropolycystic liver dz), chronic inflam involving the bile duct (PSC, liver flukes- Opisthorchis/clonorchis) and hepatolithiasis
what do intra/extrahepatic cholangiocarcinomas look like microscopically
features like adenoCA
mod differentiation and clearly defined glandular/tubular structures lined by malignant epithelial cells in abundant fibrous stroma
how do cholangiocarcinomas present
what is the prognosis
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.
what are the associatons fo angiocarcinoma
rare
association w/ vinyl chloride, arsenic and thorotrast
malignancy is rare
what are characterisitics fo hepatic lymphoma
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
what are common sites of hepatic metastasis
colon, breast, lungs and pancreas
(metastasis to the liver is more common than primary liver neoplasia)
