Cystic Lesions of Liver

Advancements in technology have brought about significant improvements in the role of medical imaging when it comes to detecting, characterizing, and managing cystic lesions affecting the liver. One noteworthy development is the utilization of imaging-guided percutaneous drainage and aspiration which has revolutionized the clinical treatment of patients dealing with cystic liver disease. 

In this detailed comparison, we explore cystic pathologies affecting the liver, shedding light on their origins, etiology, histopathological characteristics, clinical manifestations, laboratory investigations, and imaging findings. We delve into the first-line and medical management strategies, as well as potential surgical and interventional radiology interventions where applicable.

Comparing Various cystic lesion effecting Liver:

Lesion TypePrevalence/IncidenceEtiologyHistopathologyClinical FeaturesOther KeypointsUSG FeaturesCT FeaturesMRI FindingsManagement
Hepatic CystCommon (2.5-5% of population), more common in femalesDevelopmentalSerous fluid regularly outlined by cuboidal epithelium with a rim of fibrous stroma1. Often asymptomatic
2. Obstructive effect - pain, jaundice (due to biliary obstruction)
3. Other feature - early satiety, nausea and vomiting
4. Palpable mass or hepatomegaly
5. Complications like - haemorrhage and rupture can occur.
N/A1. Solitary, may be multiple
2. Well defined, round, homogeneous, anechoic with thin wall and no septations.
3. Posterior acoustic enhancement
4. Lateral wall shadow
5. Haemorrhage within cyst can appear as low level internal echoes
1. Well defined, round, homogeneous, hypoattenuating, water-density (−10 to 10 HU) lesions.
2. No wall or content enhancement after IV contrast material administration.
3. Haemorrhage within cyst can appear as High attenuation content in acute/subacute phase, and low density mass like content in chronic phase
1. T1: Very low signal
2. T2: Very high signal, increased signal intensity on heavily T2 weighted images.
3. T1 C+: No enhancement after contrast material administration.
4. Haemorrhage within cyst can appear as increased and heterogeneous signal intensity on both T1- and T2-weighted images with a fluid-fluid level.
1. Observation and Follow - up
2. Image guided Sclerotherapy
3. Surgical techniques like
- Fenestration
- Subtotal cyst excision
- Liver resection
Autosomal Dominant Polycystic Disease1 in 400 to 1 in 10001. Genetic (autosomal dominant), associated with polycystic renal disease
2. Mechanism of cyst formation - either retained abnormal bile ductules or defect in biliary cilia
1. Multiple cysts with similar histology to simple cysts
2. Altered signal pathways involving Calcium, cAMP and mTOR
1. Often asymptomatic
2. Hepatomegaly and Liver Failure in advance disease.
3. Complications like - haemorrhage and rupture can occur.
4. More common and more symptomatic in females, exogenous estrogen, and women with multiple pregnancies
1. Genetic/Molecular Markers - PKD1, PKD2 genes
2. LFTs - rarely deranged, in advance disease
1. Generally multiple
2. Well defined, round, homogeneous, anechoic
3. Haemorrhage within cyst can appear as low level internal echoes
1. Generally Multiple
2. Well defined, round, homogeneous, hypoattenuating,
3. No wall or content enhancement after IV contrast material administration.
1. T1: Very low signal
2. T2: Very high signal, increased signal intensity on heavily T2 weighted images.
3. T1 C+: No enhancement after contrast material administration.
4. Haemorrhage within cyst can appear as increased and heterogeneous signal intensity on both T1- and T2-weighted images with a fluid-fluid level.
1. Symptomatic management
2. Liver transplantation (Gigot criteria and Schnelldorfer classification)
3. Percutaneous aspiration and sclerosis
4. Open or laparoscopic cyst fenestration,
5. Combined hepatic resection and fenestration,
6. Transarterial embolisation
7. Pharmacological options:
- Somatostatin analogs (lanreotide and octreotide),
- mTOR inhibitors,
- Ursodeoxycholic acid
- Vasopressin-2 receptor antagonists
Bile Duct Hamartoma (von Meyenburg complexes)RareDevelopmental (originate from embryonic bile duct)1. Grayish-white nodular lesions, <1.5 cm, do not communicate with biliary tree
2. Irregular outline unlike hepatic cyst
Usually asymptomatic1. Size < 1.5 cm helps differentiate from, hepatic cyst / polycystic disease
2. Cystic nature of the lesions are best demonstrated on MRI
1. Multiple, Hyperechoic, well-defined, cyst like lesion scattered in bilateral liver parenchyma.
2. Size < 1.5 cm (differentiate from polycystic disease)
1. Multiple small uniformly hypoattenuating (attenuation depend on relative amount of solid to cystic component) predominantly cyst like nodules,
2. Size < 1.5 cm,
3. No enhancement (most common) to homogenous enhancement (with solid component)
1. T1: Low signal
2. T2: High signal, increased signal intensity on heavily T2 weighted images.
3. T1 C+: No enhancement (most common), homogenous enhancement (solid component)
4. MRCP: Multiple, small cystic lesions not communicating with the biliary channels
Observation
Caroli DiseaseRare1. Congenital, Autosomal recessive
2. Cavernous ectasia of bile ducts observed
Saccular dilatation of intrahepatic bile ducts1. Feature of cholangitis (Recurrent pain in right upper quadrant, Fever, Jaundice)
2. Intrahepatic biliary calculi
3. Abscess formation
4. Features of Hepatic fibrosis and portal hypertension
1. Genetic/Molecular Markers - PRKCSH, SEC63 genes
2. Multiple intrahepatic calculi
3. Associated renal cystic disease
4. Higher risk for cholangiocarcinoma
1. Dilated intrahepatic ducts with central dot
2. Colour Flow can be seen with central dot suggestive of portal vein radicle
1. Dilated and hypoattenuating cystic structures communicating with biliary tree,
2. Strong contrast enhancement (portal vein radicle) within dilated intrahepatic biliary channel "central dot" sign
1. T1: Hypointense signal
2. T2: High signal, increased signal intensity on heavily T2 weighted images.
3. T1 C+: Strong contrast enhancement (portal vein radicle) within dilated intrahepatic biliary channel
1. Antibiotics for cholangitis
2. Liver resection: hepatic lobectomy or segmentectomy depending upon the lobar / segmental involvement
3. Decompression of biliary tract
4. Liver Transplant
Undifferentiated Embryonal SarcomaRare, mainly in children/adolescentsNeoplasticLarge cystic mass, myxoid stroma, Pseudo-capsuleIHC - Desmin, vimentin positive
Limited role
1. Well defined, large (> 10 cm), solitary, predominantly cystic, occasional calcifications, Pseudo-capsule
2. Peripheral heterogenous enhancement noted on delayed images
1. T1: Hypointense signal
2. T2: Marked high signal
3. T1 C+: Peripheral heterogenous enhancement noted on delayed images.
4. Haemorrhage appear as increased and heterogeneous signal intensity on both T1- and T2-weighted image.
Surgical resection
Biliary Cystadenoma/CystadenocarcinomaVery rare, more common in femalesPre-malignant lesion (Biliary cystadenoma)1. Multilocular cystic tumors
2. Mucin-secreting cells lining the cyst wall,
3. Fluid within contains protein and mucin
1. Pain, biliary obstruction
2. >50% cases show lesion in right lobe of liver, ~ 30% in left lobe of liver and bilateral in 15% of the patients
1. IHC - MUC1, MUC5AC positive
2. Radiation therapy has Limited role
3. Cystic fluid analysis for CA-19-9 and CEA
1. Solitary, well defined, multilocular cystic mass, with thick septations1. Solitary cystic mass, thick fibrous capsule, mural nodules1. T1: Homogenous hypointense signal
2. T2: Homogenous high signal
3. Variable signals on T1 and T2 can be seen depending upon hemorrhage and protein content.
1. Surgical resection
2. Fine needle aspiration is not recommended due to risk of dissemination.
Hepatocellular Carcinoma (cystic variant)Common, especially in cirrhotic patientsNeoplasticVariable; often hypervascular solid part, capsuleSymptoms of cirrhosis, tumor-related symptoms1. Staging of Hepatocellular carcinoma
2. Performance Status
3. Objective radiological response
Features of cirrhosis and finding of a space occupying lesion (Triple phase CT is needed for characterisation of the lesion)1. Features of cirrhosis 2. LIRADS criteria (arterial enhancement with non-peripheral venous washout, capsule enhancement and evidence of interval increase in size)1. Features of cirrhosis 2. LIRADS criteria (arterial enhancement with venous washout, evidence of interval increase in size)1. Ablative therapy
2. Surgical resection
3. Liver transplant
4. TACE / TARE
5. Oral protein kinase inhibitor like sorafenib.
6. Best supportive care
HemangiomaVery commonBenign vascular lesionWell-circumscribed, red-brown, blood-filled vascular spacesOften asymptomaticPeripheral nodular enhancement in arterial phase, progressive centripetal filling in subsequent phasesHomogeneous, hyperechoicHypo-/isodense on unenhanced, peripheral nodular enhancement in arterial phase, progressive centripetal fillingHyperintense on T2, low signal on T1, characteristic enhancement pattern. Heterogeneous pattern in large sized hemangioma due to calcification, thrombus and or fibrosis.Observation unless symptomatic
Abscess (Pyogenic, Amebic, Fungal)Variable, depends on causeInfectious (bacteria, Entamoeba histolytica, Candida albicans)Inflammatory cells, necrosisSymptoms varies based on the cause of infectionRoute of Spread - Hematogenous, biliary, direct extension
Labs - CBC, culture, serology for specific infection
Appearance varies based on the cause of infectionAppearance varies based on the cause of infectionAppearance varies based on the cause of infection1. Antibiotics for 4-6 weeks but the actual duration can vary depending upon clinical response and type of infection.
2. Image guided percutaneous aspiration/drainage
3. Surgical management
Hydatid CystEndemic in sheep-raising regionsEchinococcus granulosus infectionThree layers: pericyst, endocyst, ectocystAsymptomatic , abdominal discomfort, anaphylaxis, cholangitis (Biliary Echinococcosis)Route of Spread - Ingestion of eggs
Labs - Serology, eosinophil count, LFTs
Gharbi classification and WHO classificationWell-defined, calcifications, daughter cystsHypointense rim (pericyst), Hyperintense matrix, hypointense daughter cysts1. Anti-parasitic drugs (prevention),
2. Surgical removal (curative)
3. PAIR (percutaneous aspiration, injection, reaspiration)
Intrahepatic HematomaDepends on incidence of trauma, surgeryTrauma, surgery, neoplasm (e.g., adenoma)Blood products, fibrinSymptoms depend on severity and locationN/A1. High attenuation seen in acute/subacute,
2. Fluid-like appearance in chronic
High signal in subacuteDepends on cause, severity
BilomaRare, depends on biliary injuryBiliary rupture (traumatic, iatrogenic)Bile extravasation, inflammatory reactionSymptoms depend on size, locationN/AWell-defined, cystic, no septa/calcificationsWell-defined, cystic, no septa/calcificationsWell-defined, cystic, no septa/calcificationsUSG guided aspiration/drainage
ERCP stenting / PTBD internal-external drain placement
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