Benign Neoplasms of Liver

July 6, 2024

Liver Cell Adenoma (LCA)

• Definition: Benign proliferation of hepatocytes in normal liver; lacks normal liver architecture (no bile ductules).
• Demographics:
  • Predominantly affects young females (ratio 11:1).
  • Associated with oral contraceptive pills (OCPs) and male anabolic hormones.
• Imaging:
  • CECT: Hypervascular solid lesion with heterogeneous enhancement, areas of hemorrhage, and necrosis.
  • MRI: Well-demarcated, heterogeneous mass with fat and hemorrhage.
• Biopsy:
  • Liver architecture is not preserved (no bile ducts).
  • Benign hepatocytes with glycogen and fat.
• Associated Conditions:
  • FAP (Familial Adenomatous Polyposis)
  • Glycogen Storage Diseases: Glycogenosis type 1A
  • Vascular Liver Diseases: Portal deprivation
  • Type 3 DM (Diabetes Mellitus)
  • NASH (Non-Alcoholic Steatohepatitis)
  • MASH (Metabolic Associated Steatosis Hepatitis), Obesity
  • McCune-Albright Syndrome
• Liver Adenomatosis:
  • Characterized by more than 10 adenomas.
  • HNF 1A subtype: Most common, not related to OCP, equal sex distribution, not associated with increased risk of malignancy, and has a spontaneous regression rate of 10%.

• Molecular Subtypes:

  

  • Beta-catenin:
    • Highest risk of malignant transformation.
    • Exon 3 mutation has the highest risk, followed by Exon 7/8.
  • HNF 1A:
    • Most common.
    • Best prognosis.
  • Inflammatory Adenoma:
    • Increased risk of bleeding.
    • Risk factors include size >5 cm, left lobe lesions, visualization of arteries within the lesion, and exophytic lesions.
    • Slight risk of malignancy.
  • Sonic Hedgehog Pathway:
    • Associated with obesity.
  • Complications:
  • Bleeding: Most common complication.
  • Malignant Transformation: 5-8% risk.
    • Higher risk in males of any size, beta-catenin mutation, females with lesions >5 cm.
    • AFP levels are normal in cases of HCC arising from LCA; well-differentiated and has a good prognosis.
  • Management:
  • Bleeding:
    • Hepatic artery embolization followed by resection.
  • Asymptomatic:
    • Females: Observe if <5 cm.
    • Males: Resection is recommended for any size.
  • Pregnancy:
    • Resection should be done before planned pregnancy.
  • Indications for Transplant:
    • Multiple unresectable tumors in men.
    • Large HCA with venous shunt.
    • Glycogen storage disorders.

Key Points

• Imaging:
  • CECT: Hypervascular, heterogeneous enhancement with hemorrhage and necrosis.
  • MRI: Heterogeneous mass with fat and hemorrhage; central scar is hyperintense on delayed phase.
• Pathology:
  • Non-preserved liver architecture.
  • Benign hepatocytes with glycogen and fat.
• Management:
  • Asymptomatic Females: Observe if <5 cm.
  • Asymptomatic Males: Resection required regardless of size.
  • Symptomatic: Surgery or embolization, depending on the situation.
  • Pregnancy: Resection prior to conception generally but if <5cm observation with USG surveillance can be tried
• MALIGNANT TRANSFORMATION :
  • Risk is 5-6%
  • More in men
  • Beta Catenin subtype
  • Size > 5cm
  • HCC - well differentiated, Normal AFP, Good prognosis ; are devoid of Vascular invasion or satellite nodules.
  • Adenomatosis : HNF 1A subtype, No increased risk of complication, Spontaneous regression in 10%

Management of Liver Cell Adenomas (HCA) in Women

Key Points:

• Liver Transplantation:
  • No longer indicated in women with multiple and bilobar HCAs (Dokmak et al, 2009).
• Surgical Resection:
  • Indicated for multiple HCAs only if the tumors are > 5 cm in diameter.
  • A two-step operative strategy with portal vein occlusion may be required in complex cases.
• Tumors < 5 cm in Diameter:
  • Pathologic confirmation is not mandatory for lesions < 5 cm in females.
  • Regular follow-up is sufficient as most of these adenomas remain stable, decrease in size, or even disappear over time (Dokmak et al, 2009).
• Oral Contraceptives (OCPs):
  • Discontinuation of OCPs remains necessary even after the complete regression of the tumor (Nault et al, 2013).
• Persistence of HCA > 5 cm:
  • After cessation of hormonal therapy, radical surgical management is likely justified.
• Preoperative Percutaneous Biopsy:
  • Not required in the vast majority of cases, even with suspected malignancy, as it does not typically change the therapeutic approach.
  • Indicated in the presence of a suspicious steatotic lesion > 5 cm detected by MRI in a female patient:
    • Histologic confirmation of the HNF1A subtype in this scenario may allow for conservative management with regular follow-up, given the extremely low risk of complications associated with this subtype.

Focal Nodular Hyperplasia (FNH)

• Overview
  • Prevalence: Second most common liver tumor after Hemangioma
  • Demographics: Primarily affects young women (30-50 years)
  • Etiology: Vascular malformation with a hyperplastic reaction due to increased arterial flow or portal deprivation
  • Hormone Receptors: ER positive, PR negative
  • Associated Factors: Angiopoietinins 1 and 2, TGF-β, Wnt/β-catenin signaling
  • Associations:
    • Budd-Chiari Syndrome
    • Hereditary Hemorrhagic Telangiectasia (HHT)
    • Congenital absence of portal flow
    • Portal vein thrombosis
    • Cirrhosis
  • Patients Undergoing Shunt Surgery: May develop FNH due to deprivation of portal blood flow
• Histology
  • Structure:
    • Well-circumscribed, unencapsulated
    • Central radiating scar
    • Benign hepatocytes separated by fibrous septa
    • Presence of large and dystrophic arteries
    • Normal liver architecture preserved
  • Variants:
    • Telangiectatic form
    • FNH with fat
    • FNH without fibrous scar

• Imaging Characteristics

  • Ultrasound (USG):
    • Homogeneous enhancement with central vascular supply
  • CT Scan:
    • Homogeneous enhancement on arterial phase
    • Central scar: Hypointense on T1, hyperintense on T2, strongly hyperintense on delayed phase

  • MRI:

    • Homogeneous enhancement on arterial, portal, and delayed phases
    • Central scar characteristics: Hypointense on T1, hyperintense on T2, strongly hyperintense on delayed phase
    • Hepatobiliary MRI contrast: Differentiates FNH from adenoma; isointense or hyperintense on hepatobiliary phase
    • Contrast-enhanced ultrasound (CEUS): Useful for small lesions

    

  • Biopsy:

    • Glutamine synthetase expression for confirmation
  • Clinical Features
  • Gender Ratio: Female > Male (9:1)
  • Symptoms: Often asymptomatic; may present with pain
  • Complications: Rare; telangiectatic FNH can cause complications
  • Size: Remains stable
  • Management
  • Asymptomatic:
    • No surgical intervention required
    • Stopping oral contraceptive pills (OCPs) is not necessary
    • No follow-up required as it is a benign lesion
  • Symptomatic:
    • Surgery indicated for symptomatic cases (e.g., left-sided lesions or pedunculated lesions)
    • Embolization if surgery is not feasible

MCQ 1: Not true regarding FNH?

• A. occurs in young women
• B. non-neoplastic clonal proliferation of hepatocytes
• C. lobular architecture is well preserved
• D. associated with OCP

Explanation:

• A: True; FNH typically occurs in young women (30-50 years).
• B: Not true; FNH is a benign tumor with a polyclonal regenerative process, not a clonal proliferation. Non-neoplastic lesions do not have clonal proliferation.
• C: True; the lobular architecture is well preserved in FNH.
• D: True; FNH is associated with OCP use, although there is no definitive causal relationship.

Correct Answer: B

MCQ 2: All of the following are associated with FNH except:

• A. Budd Chiari syndrome
• B. Portal vein thrombosis
• C. Glioblastoma
• D. None of the above

Explanation:

• A: True; Budd-Chiari syndrome is associated with FNH.
• B: True; Portal vein thrombosis is associated with FNH.
• C: True; FNH can be associated with glioblastoma in some cases.
• D: Not correct because A, B, and C are all associated with FNH.

Correct Answer: D

MCQ 3: Pathological features of FNH are all except:

• A. well-circumscribed encapsulated mass
• B. fibrous septae contains dystrophic arteries
• C. focal staining with glutamine synthetase
• D. Upregulation of angiopoietin gene

Explanation:

• A: Not true; FNH is well-circumscribed but not encapsulated.
• B: True; fibrous septa contain dystrophic arteries.
• C: True; FNH shows focal staining with glutamine synthetase.
• D: True; there is upregulation of angiopoietin genes in FNH.

Correct Answer: A

MCQ 4: Imaging features of FNH are all except:

• A. Heterogeneous arterial enhancement with central scar
• B. Absence of calcification
• C. Central scar is hyperintense on delayed phase
• D. CE-MRI is the investigation of choice

Explanation:

• A: Not true; FNH typically shows homogeneous arterial enhancement and a central scar.
• B: True; there is no calcification in FNH.
• C: True; the central scar is hyperintense on delayed phase MRI.
• D: True; Contrast-enhanced MRI (CE-MRI) is the investigation of choice for FNH.

Correct Answer: A

MCQ 5: A 35-year-old woman was incidentally diagnosed with liver SOL on USG. On CECT, features were of a 6.5*8 cm arterial enhancing lesion with a central scar in the left lobe compatible with FNH. She is on OCP for the last 4 years. What should be the further management?

• A. Surgery
• B. Embolization
• C. Follow up, stop OCP
• D. No treatment

Explanation:

• For asymptomatic FNH, no treatment is required regardless of the size. OCP use does not need to be discontinued, and no follow-up is required.

Correct Answer: D

MCQ 6: In the above question, if the patient was symptomatic with liver SOL, what would have been the preferred treatment?

• A. Enucleation
• B. Left hepatectomy
• C. Embolization
• D. RFA

Explanation:

• A: Enucleation is avoided due to the risk of bleeding and difficulty in finding a plane since FNH lacks a capsule.
• B: Preferred for symptomatic FNH, especially with parenchyma-preserving resection.
• C: Embolization may be considered if surgery is not feasible.
• D: Radiofrequency ablation (RFA) is used in select cases.

Correct Answer: B

Hemangioma

Overview

• Most Common Benign Lesion of the Liver
• Gender Prevalence: Female > Male
• Type: Vascular malformation (Cavernous Hemangioma, Focal Nodular Hyperplasia (FNH))
• Nature: Benign; typically equal distribution in both lobes

Clinical Features and Complications

General Characteristics

• Typically Asymptomatic:
  • Often discovered incidentally during laparotomy, autopsy, or routine imaging for unrelated reasons.

Symptoms in Giant Hemangiomas

• Abdominal Pain and Discomfort: More common in patients with giant hemangiomas.
• Mass Effects:
  • Early Satiety: Due to gastric compression.
  • Biliary Stasis.
  • Vascular Obstruction.

Rare Complications

• Rupture:
  • Extremely rare, even in very large hemangiomas or during pregnancy.
• Thrombosis:
  • Can lead to an inflammatory reaction, resulting in:
    • Abdominal pain.
    • Slight fever.
    • Weight loss.
    • Anemia.
    • Thrombocytosis.
    • Increased fibrinogen levels.

Kasabach-Merritt Syndrome (Adult)

• Local Consumptive Coagulopathy:
  • Caused by intravascular coagulation, clotting, and fibrinolysis within the hemangioma.
  • Can lead to a secondary increase in systemic fibrinolysis and thrombocytopenia.
• Systemic Disseminated Intravascular Coagulation (DIC):
  • Associated with a high mortality (30%-40%) due to uncontrollable bleeding.
  • Reversible by surgical removal of the hemangioma.
• Intratumoral Hemorrhage is rarely encountered in hepatic hemangioma. It can occur spontaneuosly or after anticoagulation therapy.
• Spontaneous Rupture may occur rarely in large adult hemangiomas
• In Children:
  • Can cause congestive cardiac failure (CCF)

Imaging Characteristics

• Ultrasound (USG):
  • Hyperechoic mass with sharp margins
  • Acoustic enhancement
  • No vascularity on Doppler

• CT Scan:

  • Low attenuation on non-contrast CT
  • Peripheral nodular and globular enhancement ("peripheral puddles")
  • Centripetal filling
  • Contrast enhancement on delayed phase

  

• MRI:

  • Hypointense on T1 and hyperintense on T2
  • Light bulb appearance on T2-weighted MRI
  • T2 with fat suppression
  • Giant hemangioma- thrombosis, fibrosis, hyalinazation

  

• Additional Information

  • Hormone Receptors: May be present (ER, PR, androgens) = accelerated growth seen with High estrogen states like puberty, pregnancy, OCP use, androgen treatment.
  • Size:
    • >10 cm considered giant hemangioma [ blumgart]
  • Structure:
    • Cavernous vascular spaces with septa lined by flattened endothelium.
  • Growth:
    • Some large hemangiomas may grow over time
  • Symptoms:
    • Mostly asymptomatic
    • Pressure effects from large size
    • Inflammation (low-grade fever, increased ESR, thrombocytosis)
    • Normal total leukocyte count (TLC) and liver function tests (LFT)
• Management
  • Asymptomatic: Observation irrespective of size. you dont have to stop OCP’s
  • Symptomatic (e.g., rupture, change in size, Kasabach-Merritt Syndrome):
    • Resection
  • Additional Treatments: Radiation, embolization

Liver Hemangioma: Case and Management

Patient Summary

• 35-year-old female on OCP (Oral Contraceptive Pills).
• Underwent routine USG during a physical examination.

USG Findings

• Hyperechoic lesion measuring 10 x 5 cm.
• Posterior acoustic enhancement.
• Sharp margins.

Diagnosis

• Liver Hemangioma.

Management Options

• A) Observation (Correct Answer).
• B) Stop OCP.
• C) Surgery.
• D) Embolization.

Rationale

• Hemangiomas can often be unequivocally identified by imaging studies due to their typical radiographic features.
• On USG:
  • Appears as a well-defined hyperechoic mass.
  • Acoustic enhancement and sharp margins.
• Atypical appearances may occur in larger lesions due to calcifications or thrombi.
• Fatty liver may make the lesion appear hypoechoic compared to surrounding parenchyma.
• If standard ultrasound is inconclusive:
  • Contrast-enhanced examinations like ce-US, ce-CT, and MRI are needed.
  • MRI is the best imaging modality, showing hypointensity on T1-weighted sequences and hyperintensity on T2.
• Scintigraphy with technetium 99m pertechnetate-labeled erythrocytes can document gradual accumulation of red blood cells inside the hepatic lesion.
• Hepatic angiography shows a "cotton wool" appearance but is rarely used.
• Percutaneous biopsy has a low diagnostic yield and carries the risk of severe bleeding, so it is generally avoided.

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Simple Hepatic Cyst

• Definition: Benign cystic lesions originating from the biliary tree or peribiliary glands.
• Histology:
  • Lined by simple cuboidal epithelium.
  • Contains serous fluid.
  • No communication with the biliary tree.
• Demographics:
  • Higher incidence in adults.
  • More common in females.
• Imaging Characteristics:
  • Ultrasound (USG):
    • Anechoic (fluid-filled), well-defined.
    • No septations or nodularity.
  • MRI:
    • Low signal intensity on T1-weighted images.
    • Bright signal intensity on T2-weighted images.
    • Non-enhancing on contrast-enhanced MRI.
  • CT Scan:
    • Non-enhancing, cystic appearance.
• Treatment:
  • Asymptomatic Cysts: No treatment required.
  • Image-Guided Aspiration:
    • Not recommended due to a 100% recurrence rate.
  • Aspiration + Sclerosant:
    • Used if biliary communication is ruled out.
  • Cyst Fenestration/Reection:
    • Fenestration is preferred to reduce recurrence (10% recurrence rate).
  • Laparoscopic Fenestration:
    • Treatment of choice due to minimally invasive nature and reduced recurrence rates.

Summary

Simple hepatic cysts are benign, fluid-filled lesions with no biliary communication, typically managed conservatively if asymptomatic. Imaging characteristics are consistent across modalities, and recurrence is a concern with non-surgical interventions. Laparoscopic fenestration is the preferred treatment option for symptomatic cases.

Polycystic Liver Disease (PLD)

• Definition: Genetic disorder characterized by the presence of multiple cysts in the liver, which can be associated with autosomal dominant polycystic kidney disease (ADPKD).
• Demographics:
  • More common in women.
  • Pregnancy and hormone intake may exacerbate symptoms.
• Genetics:
  • ADPKD: Involves PKD1 and PKD2 genes, which primarily affect the kidneys and Brain [but can also have liver cysts].
    • PKD1 and PKD2 predict the severity of renal cysts but not liver cysts.
  • Autosomal Dominant Polycystic Liver Disease (ADPLD): Caused by PRKCSH or SEC63 gene mutations.
• Clinical Features:
  • Often asymptomatic.
  • Liver dysfunction is rare.
  • Potential complications include:
    • Infection
    • Rupture
    • Portal hypertension
    • Venous outflow obstruction
• Classification:
  • Gigot Classification:
    • Type 1: 10 or fewer large cysts (>10 cm) with significant areas of non-involved liver parenchyma.
    • Type 2: Diffuse involvement by medium-sized cysts with large areas of non-cystic liver parenchyma.
    • Type 3: Massive and diffuse cystic involvement with only a few areas of normal liver substance.

• Imaging:

  • CT Scan and MRI are used for diagnosis and classification of liver cysts.

    

• Treatment Options:

  • Medical Management:
    • Somatostatin Analogues: May reduce cyst growth.
    • Sirolimus: Can help in managing symptoms and slowing disease progression.
  • Procedural Interventions:
    • Cyst Aspiration + Sclerosant Injection: Used to manage symptoms in some cases.
    • Cyst Fenestration: Effective for Type 1 PLD.
    • Hepatic Artery Embolization: Can be used for symptomatic relief.
  • Surgical Management:
    • Type 3 PLD: Liver transplantation, possibly combined with kidney transplantation.
• Complications:
  • Ascites: Most common complication.
  • Other complications include infection, rupture, portal hypertension, and venous outflow obstruction.

Summary

Polycystic liver disease is associated with both genetic and hormonal factors, often presenting with multiple liver cysts. The condition can be classified into three types based on the extent of liver involvement. Management varies from medical therapies and procedural interventions to surgical options, particularly for more severe cases.

Neoplastic Cysts Overview

Types of Neoplastic Cysts

• Primary Cystic Neoplasia
• Cystic Metastases
• Cystic Degeneration of Solid Liver Metastases

Biliary Cystadenoma

• Similar to: Mucinous cystic neoplasm of the pancreas.
• Epidemiology:
  • 90% occur in women.
  • Cystadenocarcinoma has an equal gender distribution (M=F).
• Characteristics:
  • May communicate with the biliary tree.
  • Contains ovarian stroma that expresses Estrogen Receptor (ER) and Progesterone Receptor (PR).
  • Embryologic origin from ectopic ovarian tissue.

Intraductal Papillary Mucinous Neoplasm (IPMN) of the Bile Duct

• Similar to: IPMN of the pancreas.
• Characteristics:
  • Contains papillary projections that secrete mucin.
  • Presents as a diffusely dilated bile duct or a large cystic mass.
• Invasive Cancer:
  • Found in 60% of specimens.
  • Better prognosis than standard bile duct cancer.
  • 5-year survival: 60-80%.
• Imaging (USG/CT/MRI):
  • Shows enhancing septation, mural nodularity, and papillary projections.
  • CEA (Carcinoembryonic Antigen) may be elevated.
• Treatment:
  • Complete surgical excision is recommended.

Liver Neoplasms Imaging Findings

Neoplasm	NCCT Findings	Early Arterial Phase CT Findings (15s)	Late Arterial Phase CT Findings (30s)	Portal Venous Phase CT Findings (60s)	Delayed Venous Phase CT Findings (3-5m)	T1 MRI Findings	T2 MRI Findings	MRI Findings	Miscellaneous
FNH	Similar to parenchyma		Hyper, homogenous	Iso to Hyper	iso	Hypo	hyperintense	Similar to parenchyma	Kupffer and Bile ducts +
Central Scar (FNH)	Hypoattenuating scar	Not specified	Hypo	Hypo	Hyper, Scar enhancement	Hypointense	Hyperintense	Hypoattenuating scar	central stellate scar = FNH, FL HCC -
SCN pancreas -
Kidney oncocytoma -
Adenoma	Similar to parenchyma	Not specified	Moderate Enhances-
starts peripherally Not nodular and progress Centripetally	Similar to Parenchyma	Iso dense	hyper - Isointense	HYper slightly
Hemangioma	Hypo, well defined		Peripheral nodular enhancement	fills centripetally	Persistence due to pooling	Hypointense	Hyperintense = LIght Bulb sign	Peripheral nodular enhancement
Biliary cystadenoma	Multilocular, defined cystic lesion	Wall and septae get enhanced	Wall and septae get enhanced	Not specified	Not specified	Hypointense	Hyperintense	Wall and septae get enhanced
Biliary cystadeno Ca	Thick irregular wall	Thick irregular wall	Not specified	Rim enhancement ; mural nodule	Not specified	Hypointense	Hyperintense	Thick irregular wall
HCC (Hepatocellular Carcinoma)	Not visible≈ Adenoma	Hyper, homogenous enhance	Not specified	Not specified	Not specified	Hypointense	Hyperintense	Hyper, homogenous enhancement
Fibrolamellar HCC	Not specified	Hyper, homogenous enhance	Not specified	Not specified	Not specified	Hypointense	Hyperintense	Hyper, homogenous enhancement
Cholangiocarcinoma	Not specified	Not specified	Not specified	Not specified	Not specified	Hypointense	Hyperintense	Not specified
Metastatic Lesions	Hypovascular: Hypo, Hypervascular: Hyper	Hypovascular: Not specified, Hypervascular: Hyper	Not specified	Not specified	Not specified	Variable	Variable	Hypovascular: Hypo, Hypervascular: Hyper

LCA MCQ’s

MCQ: Identify the False Statement

Options:

• A) Marked steatosis is seen in HNF1A subtype of HCA.
• B) bHCA is associated with pseudoglandular formation and atypia.
• C) IHCA subtype is more common with NASH.
• D) Biopsy is mandatory for molecular subtype of HCA.

Correct Answer: D) Biopsy is mandatory for molecular subtype of HCA.

Explanation:

• A) True: Marked steatosis is characteristic of the HNF1A subtype of Hepatocellular Adenoma (HCA).
• B) True: bHCA (Beta-catenin activated HCA) is associated with pseudoglandular formation and atypia, and has a higher risk of malignant transformation.
• C) True: IHCA (Inflammatory Hepatocellular Adenoma) is indeed more commonly associated with Non-Alcoholic Steatohepatitis (NASH).
• D) False: Biopsy is not always mandatory to determine the molecular subtype of HCA, as non-invasive imaging and specific clinical features can often be sufficient for diagnosis.

MCQ: Identify the Incorrect Risk Factor for Liver Cell Adenoma

Options:

• A) After shunt surgery
• B) Obesity
• C) NASH
• D) Wilson disease

Correct Answer: D) Wilson disease

Explanation:

• A) After shunt surgery: True—Shunt surgery can be a risk factor due to changes in hepatic blood flow.
• B) Obesity: True—Obesity is a known risk factor for the development of liver cell adenomas.
• C) NASH (Non-Alcoholic Steatohepatitis): True—NASH is associated with an increased risk of liver cell adenoma.
• D) Wilson disease: False—Wilson disease is not a recognized risk factor for liver cell adenoma. It is a genetic disorder related to copper metabolism, leading to different hepatic issues such as cirrhosis, but not specifically liver cell adenomas.

MCQ: Identify the False Statement Regarding Imaging in Liver Cell Adenoma(LCA)

Options:

• A) CE MRI is the investigation of choice
• B) Atoll sign is seen in inflammatory subtype of LCA
• C) Can be differentiated from HCC on triphasic CT
• D) Diffuse and homogenous signal dropout on T1 weighted sequence is the most striking feature

Correct Answer: C) Can be differentiated from HCC on triphasic CT

Explanation:

• A) True: Contrast-enhanced MRI (CE MRI) is the investigation of choice for diagnosing and characterizing liver cell adenomas.
• B) True: The Atoll sign (a peripheral ring of high signal intensity) is associated with the inflammatory subtype of liver cell adenomas.
• C) False: Differentiating liver cell adenoma from Hepatocellular Carcinoma (HCC) on triphasic CT can be challenging, as both can present with similar imaging characteristics, particularly in their arterial phase enhancement patterns.
• D) True: Diffuse and homogenous signal dropout on T1-weighted sequences is indeed a striking feature, often seen due to the fat content in liver cell adenomas.

MCQ: Identify the Incorrect Risk Factor for Hemorrhage in Hepatocellular Adenoma (HCA)

Options:

• A) Male gender
• B) Large tumors > 5 cm
• C) Location in left lobe
• D) Exophytic growth

Correct Answer: A) Male gender

Explanation:

• A) Male gender: False—Male gender is not considered a specific risk factor for hemorrhage in HCA. However, HCAs are generally more common in females, especially those on oral contraceptives.
• B) Large tumors > 5 cm: True—Larger HCAs (greater than 5 cm especially in IHCA subtype) have a higher risk of hemorrhage.
• C) Location in left lobe: True—HCAs located in the left lateral lobe are more prone to complications, including hemorrhage.
• D) Exophytic growth: True—Exophytic growth, where the tumor grows outward from the liver, is associated with an increased risk of rupture and hemorrhage.
• Visualisation of arteries within the lesion is also a risk factor

Case Summary

Patient: 35-year-old female

Presentation:

• Hypovolemic shock
• CT Scan Findings: 6 x 4 cm hyperenhancing, heterogeneous enhancing SOL (space-occupying lesion) in the right lobe of the liver with hemoperitoneum.
• Lab Results:
  • Hemoglobin (Hb): 6.5 g/dL
  • TLC (Total Leukocyte Count): 15,000 cells/µL
  • Platelets: 150,000/µL

Management Options:

• A) Proceed for surgery
• B) Embolization (Correct Answer)
• C) RFA (Radiofrequency Ablation)
• D) Observation alone

Correct Answer: B) Embolization

Rationale

• Diagnosis: The patient likely has a liver cell adenoma (LCA) with hemorrhage, as indicated by the hyperenhancing, heterogeneous lesion on CT and associated hemoperitoneum.
• Management:
  • First Line: Embolization is the preferred initial treatment to control the bleeding and stabilize the patient. It effectively reduces the blood supply to the hemorrhaging lesion, minimizing further blood loss.
  • Next Step: After successful embolization and stabilization, surgery may be considered for definitive treatment if necessary, such as resection of the adenoma to prevent recurrence or further complications.
• Other Options:
  • Surgery (A): Immediate surgery in the setting of active bleeding and hypovolemic shock is generally avoided unless absolutely necessary, as embolization can stabilize the situation with less risk.
  • RFA (C): Radiofrequency ablation is not suitable for managing hemorrhage and is typically used for small, localized liver tumors.
  • Observation (D): Observation alone is inappropriate in the setting of hypovolemic shock and active bleeding.

MCQ: Identify the False Statement Regarding Malignant Degeneration of Hepatocellular Adenoma (HCA)

Options:

• A) Well differentiated
• B) Size > 5 cm
• C) AFP > 500
• D) Better prognosis than spontaneous HCC

Correct Answer: C) AFP > 500

Explanation:

• A) Well differentiated: True—Malignant degeneration of HCA into hepatocellular carcinoma (HCC) often presents as well-differentiated tumors.
• B) Size > 5 cm: True—Larger HCAs (>5 cm) are at higher risk for malignant transformation.
• C) AFP > 500: False—Alpha-fetoprotein (AFP) is typically normal in HCA, and an AFP level >500 ng/mL is more indicative of primary hepatocellular carcinoma (HCC) rather than malignant transformation of HCA.
• D) Better prognosis than spontaneous HCC: True—Malignant degeneration of HCA generally has a better prognosis compared to spontaneous HCC due to earlier detection and better differentiation.

Case Summary

Patient: 32-year-old female

Presentation: Consultation for a 4 cm liver cell adenoma (LCA) in the left lobe.

Future Plan: The patient wants to plan for pregnancy.

Management Options:

• A) Resection of adenoma before pregnancy
• B) Embolization
• C) Can go ahead with pregnancy with frequent USG surveillance (Correct Answer)
• D) None of the above

Correct Answer: C) Can go ahead with pregnancy with frequent USG surveillance

Rationale

• Hormonal Considerations:
  • There is a concern regarding hormone-induced growth and potential rupture of the adenoma during pregnancy due to increased levels of estrogen and progesterone.
• Evidence:
  • Studies have shown that most HCAs remain stable during pregnancy, particularly if they are less than 5 cm in diameter and there is no evidence of intratumoral bleeding.
• Recommendation:
  • Pregnancy can be considered safe in women with HCAs smaller than 5 cm, provided that frequent ultrasound (USG) surveillance is conducted throughout the pregnancy to monitor the size and characteristics of the adenoma.
• Other Options:
  • Resection (A) or Embolization (B) may be considered for larger or symptomatic adenomas, but they are not necessary for this patient based on current evidence.
  • None of the above (D) is not appropriate since there is a suitable management plan (C).

MCQ: Identify the Incorrect Indication for Surgery in Liver Cell Adenoma (LCA)

Options:

• A) Size > 5 cm in HNF1A subtype, female gender
• B) Size < 5 cm, Male gender, HNF1A subtype
• C) Size > 5 cm, Female gender, OCP use
• D) Multiple and bilobar HCA
• E) Both A and D

Correct Answer: E) Both A and D

Rationale

• A) Size > 5 cm in HNF1A subtype, female gender:
  • False: Surgery is generally not indicated for HNF1A subtype adenomas, especially in females, unless other risk factors (e.g., large size, symptoms, or failure to regress after stopping OCPs) are present. HNF1A adenomas have a very low risk of complications, so conservative management with regular follow-up is preferred.
• B) Size < 5 cm, Male gender, HNF1A subtype:
  • True: Surgery is generally considered in male patients with HCAs due to a higher risk of malignant transformation, even for smaller lesions. However, the HNF1A subtype is less concerning, but male gender still increases the risk.
• C) Size > 5 cm, Female gender, OCP use:
  • True: Surgery is indicated for large adenomas (>5 cm) in females, especially if they are on oral contraceptives (OCPs), due to the risk of hemorrhage and malignant transformation.
• D) Multiple and bilobar HCA:
  • False: Multiple and bilobar HCAs do not necessarily require surgery, especially if they are <5 cm in size. Surgery is typically reserved for larger lesions or if the adenomas fail to regress after discontinuation of OCPs.
• E) Both A and D:
  • Correct Answer: Both A and D are not absolute indications for surgery in LCAs. Conservative management with regular follow-up is often the preferred approach, particularly in low-risk cases like HNF1A subtype adenomas in females and multiple small lesions.

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FNH MCQ’s

MCQ: Identify the False Imaging Feature of Focal Nodular Hyperplasia (FNH)

Options:

• A) Lack of capsule
• B) Homogenous lobulated appearance
• C) Hyperenhancing central scar on arterial phase
• D) Absence of necrosis, hemorrhage, and calcification

Correct Answer: C) Hyperenhancing central scar on arterial phase

Explanation:

• A) Lack of capsule: True—FNH typically lacks a capsule.
• B) Homogenous lobulated appearance: True—FNH usually has a homogenous lobulated appearance on imaging.
• C) Hyperenhancing central scar on arterial phase: False—The central scar in FNH typically enhances on delayed phase rather than during the arterial phase.
• D) Absence of necrosis, hemorrhage, and calcification: True—FNH usually does not show necrosis, hemorrhage, or calcification on imaging.

Imaging Features of Focal Nodular Hyperplasia (FNH)

• Lack of Capsule:
  • FNH typically does not have a capsule.
• Lobulated Contours:
  • FNH often exhibits lobulated contours on imaging.
• Lesion Homogeneity:
  • The lesion is generally homogeneous except for the central scar.
• US, CT, and MRI Characteristics:
  • Echogenicity on ultrasound, CT attenuation, and MRI signal intensity are similar or slightly different from the adjacent liver on precontrast imaging.
• Arterial Phase Enhancement:
  • FNH shows strong and homogeneous enhancement on the arterial phase of contrast-enhanced ultrasound (CEUS), CT, or MRI.
  • The lesion becomes similar to the adjacent liver on portal and delayed phases.
• Central Scar:
  • Best visualized on MRI:
    • Hypointense on precontrast T1-weighted images.
    • Strongly hyperintense on T2-weighted images.
    • Becomes hyperintense on delayed phase due to contrast accumulation in the fibrous tissue.