Pathological Conditions of the Liver Pt 2

how do you get glycogen storage disease

inherited disease

_____ is an abnormal storage and accumulation of gylcogen in the tissues, especially the liver and kidneys

glycogen storage disease

what is the most common type of glycogen storage disease

von Gierke/type I (in the liver and kidneys)

glycogen storage disease is associated with what 4 things

1. hepatic adenomas

2. FNH (focal nodular hyperplasia)

3. hyperplasia

4. hepatomegaly

what is the sonography for glycogen storage disease

hepatomegaly, increased echogenicity, and slightly increased attenuation

what is the sonography for adenomas

well-demarcated, round, homogenous, echogenic tumors

are adenomas benign or malignant

benign

_____ is when there is excess iron deposits throughout the body (iron metabolism disease)

hemochromatosis

what is the sonography of hemochromatosis

hepatomegaly, cirrhotic changes, increased echogenicity may be seen

______ develops secondary to CHF with signs of hepatomegaly

passive hepatic congestion

with CHF the IVC and hepatic veins fill with more blood which causes _____ in the liver

congestion

simple/congenital cysts (non parasitic) are seen more often in ____

women

peribiliary cysts range in size from _____

0.2 - 2.5 cm

peribiliary cysts are more commonly found in patients with ________

severe liver disease

where are peribiliary cysts located

centerally within porta hepatis at junction of rt and lt hepatic ducts

how do you get polycystic liver disease

Inherited (in an autosomal dominant)

polycystic liver diease is most common in ___

middle aged women

at least ___% of pts with polycystic renal disease have one to several hepatic cysts

50-74%

___% of those with polycystic liver disease also have associated polycystic renal disease

60%

what size are the cysts that occur with polycystic liver disease

less than 2-3 cm

what are the labs associated with polycystic liver disease

normal LFTs

what are the labs associated with simple/congenital cysts

normal LFTs

what does pyogenic mean

pus forming

inflammatory disease of the liver often comes from what

intestinal source, appendicitis, diverticulitis, or cholecystitis

what are the 3 basic location types for abscess formation in the liver

intrahepatic, subhepatic, and subphrenic

what are the clinical symptoms associated with inflammatory disease of the liver

1. fever

2. elevated WBC count

3. RUQ pain

what is the sonography for pyogenic abscess/ inflammatory lesions

1. solitary or multiple lesions

2. abnormal fluid collections in morrisons pouch

3. fluid filled, varying echoes, irregular borders, gas, or calcification

how would an infection in the GI tract get to the liver

portal venous system

what location is the most common site for pyogenic abscesses to occur

rt lobe of the liver

hepatic candidiasis (fungal abscess) is caused by a species of _____

candida

______ occurs in immunocompromised hosts, such as patients undergoing chemotherapy, organ transplant recipients, or individuals with human immunodeficiency infection (HIV)

hepatic candidiasis

what sonographic sign is associated with candidiasis abscesses

bull’s eye (target) lesions

_____ is a congenital defect in immune system cells called phagocytes that renders the cells unable to kill ingested bacteria, resulting in increased susceptibility to severe infections

chronic granulomatous disease

chronic granulomatous disease mostly is seen in _____

children (mostly girls)

what is the sonography of chronic granulomatous disease

poorly marginated, hypoechoic mass seen with posterior enhancement, calcifications may be present with posterior shadowing

echinococcal cysts are common in what community

sheep herding

echinococcus is a ____

tapeworm that infects humans

where does the echinococcus worm reside

small intestine of dogs

what are the clinical symptoms associated with hydatid/echinococcal cysts

asymptomatic, RUQ pain, hepatomegaly

what test is used to diagnose hydatid disease

casoni test

what is the sonography for hydatid / echinococcal cysts

two layers: outer inflammatory layer and inner daughter cysts. multilocular apperance, thick walls, can appear simple

_____ is the most common organism causing opportunistic infection in patients with acquired immunodeficiency syndrome.

pneumocytis carinii

pneumocystis carinii is a common life-threatening infection in patients with _____

HIV

_____ affects patients undergoing bone marrow and organ transplantation or patients receiving chemotherapy

pneumocystis carinii

what is the sonography for pneumocystis carinii

ranges from diffuse, tiny, nonshadowing echogenic foci to extensive replacement of the liver parenchyma by various echogenic clumps of calcification

_____ is a collection of pus formed by disintegrated tissue in a cavity caused by parasite Entamoeba histolytica

amebic abscess

Originates in the GI tract and the amebic parasite reaches liver via the _____

portal vein

how is the entamoeba histolytica parasite contracted

via ingesting contaminated water and food

what are the symptoms for amebic abscess

1. asymptomatic

2. GI symptoms, abdominal pain, diarrhea, leukocytosis, low fever

what is the sonography for amebic abscess

variable and nonspecific, round or oval, may lack significantly defined wall echoes, may have some internal echoes along posterior margin secondary to debris, distal enhancement may be seen

____ are benign congenital tumors consisting of small to large blood filled cystic spaces

cavernous hemangioma

what is the most common tumor of the liver

cavernous hemangioma

what group of people are cavernous hemangiomas commonly found in

women

what are the clinical symptoms of cavernous hemangiomas

asymptomatic

what are the lab values associated with cavernous hemangioma

normal

what is the sonography for cavernous hemangiomas

hyperechoic with acoustic transmission, round/oval/lobulated with well defined borders, larger may be more heterogenous due to necrosis, some may bleed with age

what is the aging process for cavernous hemangiomas

degeneration > fibrosis > calcification

as a hemangioma ages, it may become more _____

heterogenous

________ is a tumor of the glandular epithelium in which the cells of the tumor are arranged in a recognizable glandular structure

adenoma

______ consists of normal or slightly atypical hepatocytes and frequently contains areas of bile stasis and focal hemorrhage or necrosis

liver cell adenoma

liver cell adenoma is common with _______

women on birth control pills but can happen in men.

what are the clinical symptoms for liver cell adenoma

palpable abdominal mass, RUQ pain, secondary to rupture with bleeding into the tumor

what lab values are associated with liver cell adenoma

normal

what is the sonography for liver cell adenomas

well defined solid mass that is homogenous and often hypoechoic, can become heterogenous due to central hemorrhage or necrosis which can give it a hypoechoic center (might mimick FNH), solitary or multiple

what pathology is associated with birth control pills

liver cell adenoma

while it doesnt cause it, birth control pills can make EXISTING _____ grow

FNH

incidence for liver cell adenoma is increased in patients with ________

von gierke disease (type I glycogen storage disease)

_____ masses are thought to arise from developmental hyperplastic lesions related to an area of congenital vascular formation

FNH (focal nodular hyperplasia)

what is the second most common tumor to cavernous hemangioma

focal nodular hyperplasia (FNH)

FNH is most common in what group

women under 40

what is the clinical symptoms of FNH

asymptomatic unless mass effect

what lab values are associated with FNH

normal

FNH is common in the ___ lobe of the liver

right

what is the sonography for FNH

well defined hyperechoic to isoechoic to liver parenchyma, central stellate scar usually a dominate artery that feeds the mass

what is the most common symptomatic vascular tumor in infancy

infantile hemangioendothelioma

______ is an overgrowth of the endothelium of minute capillary vessels that occurs in infants

infantile hemangioendothelioma

____% of infantile hemangioendotheliomas occur befor 6 months of age

85%

what is the sonography associated with infantile hemangioendotheliomas

hypoechoic lesions, large draining veins and dilated prox abdominal aorta with AV shunting

____ is a rare, developmental, unencapsulated, cystic tumor of the liver

mesenchymal hamartoma

what are the components of a mesenchymal hamartoma

bile ducts, primitive mesenchyma, and hepatic parenchyma with stromal/cystic elements

what is the sonography for a mesenchymal hamartoma

well defined complex mass, 5-30cm, most common in rt lobe

___% of patients with preexisting cirrhosis develop hepatocellular carcinoma

80%

hepatocellular carcinoma is more commonly found in ___

men

the pathogenesis of HCC is often related to ____

cirrhosis, chronic hep B, and hepatocarcinogens in foods

what is the clinical presentation of HCC

1. history of cirrhosis or hep B/C

2. palpable mass

3. hepatomegaly

4. jaunice

5. weight loss

6. portal HTN, ascites, splenomegaly

what lab values are associated with HCC

elevated AFP, eleavted alk phos

what is the sonography for HCC

1. variable sonography

2. can be very invasive

3. solitary large tumor, multiple nodules throughout, diffuse infiltrative masses

4. hypoechoic, isoechoic, hyperechoic, or infiltrating

5. may invade hepatic veins and portal veins

6. tendency to destroy the portal venous radicle walls with invasion into the lumen of the vessel

7. may have necrosis and hemorrhage

what is the most common form of neoplastic involvement of the liver

metastatic disease

what organs do metastatic disease usually originate from

colon, breast, lung

what are the clinical symptoms of metastatic disease

RUQ pain, weight loss, hepatomegaly, splenomegaly

what lab values are associated with metastatic disease

elevated alk phos, elevated serum bilirubin

what is the sonography for metastatic disease

varies. Hypoechoic or echogenic mass, diffuse distortion of BULLS EYE pattern, solitary or multiple well to ill-defined lesions

what is the most common liver tumor that occurs in infancy

hepatoblastoma

what is the clinical presentation of hepatoblastomas

abdominal enlargement, weight loss, hepatomegaly, precocious puberty

what lab values are associated with hepatoblastoma

elevated AFP

what is the sonography of hepatoblastomas

heterogenous, hyperechoic mass or cystic with septations, possible calcifications

______ is a malignant neoplasm involving lymphocyte proliferation in the lymph nodes

lymphoma

what are the 2 main types of lymphoma

Hodgkin and non-Hodgkin lymphoma

how are Hodgkin lymphoma and non-Hodgkin lymphoma differentiated

by lymph node biopsy

what is the clinical presentation for lymphoma

1. hepatomegaly

2. normal or diffuse alteration of parenchymal echoes

3. usually present with the disease elsewhere

4. enlarged, nontender lymph nodes

5. fever, fatigue, night sweats, weight loss, bone pain, or an abdominal mass