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6. What is the cause of steatorrhea in a patient with Crohn’s disease? A. Excess excretion of bilirubin and cholesterol in stool B. Concurrent obstructive primary sclerosing
cholangitis
C. Terminal ileum resections resulting in bile acid and
salts malabsorption
D. Intestinal bacterial depletion resulting in inadequate
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bilirubin conjugation
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7. In your patient taking 5 mg of warfarin daily for a pros­thetic mitral valve, how might metronidazole treatment for Clostridium difficile affect their international normal­ized ratio (INR)? A. Increase the INR due to cytochrome P-450 isoform
inhibition. B. Increase the INR due to induction of P-450 isoform. C. No change, metronidazole does not affect hepatic
metabolism of P-450 isoforms. D. Reduction of INR due to alterations in intestinal
microflora.
Answer: C
Bile salts are sodium and potassium salts of bile acids derived from cholesterol by hepatocytes. After synthesis, the primary bile acids cholic and chenodeoxycholic acid are conjugated to either taurine or glycine and then secreted into the biliary system. Approximately 90%–95% of these primary bile salts and acids are absorbed by active transport at the terminal ileum, a region commonly affected by Crohn disease, while the remainder enter the colon and are converted to second­ary bile acids (deoxycholic and lithocolic acids) and their associated salts by resident bacteria. Bile acids and salts reabsorbed in the terminal ileum are reabsorbed through the portal circulation, while those lost in the stool are replaced by hepatic synthesis. The continuous process of secretion of bile salts in the bile, their passage through the intestine, and their subsequent return to the liver is termed the enterohepatic circulation. (See Schwartz 11th ed., p. 1352.)
Answer: A
Many factors can affect drug metabolism in the liver. When the rate of metabolism of a drug is increased (ie, enzyme induction), the duration of the drug action will decrease. However, when the metabolism of a drug is decreased (ie, enzyme inhibition), then the drug will circulate for a longer period of time, potentially increasing the effect of the medica­tion. Warfarin is a vitamin K antagonist and therefore reduces hepatic synthesis of coagulation factors. As metronidazole inhibits the metabolism of warfarin, the anti-coagulation effects are increased and the INR may rise. (See Schwartz 11th ed., p. 1352.)
8. Which of the following compounds is not synthesized predominantly by the liver? A. Albumin B. Factor VIII C. Factor VII D. Factor II
9. What technique is the most sensitive for the identifica­tion of liver lesions? A. Intraoperative ultrasound B. Computed tomography (CT) with triple-phase
contrast
C. Magnetic resonance imaging (MRI) with gadoxetate-
based contrast
D. Abdominal ultrasound
Answer: B
The liver is the largest gland in the body, and responsible for synthesis of the majority of plasma proteins. The liver pro­duces approximately 10 g of albumin per day, and albumin measurement can therefore be used as a surrogate for liver synthetic function. This must be interpreted with caution, as albumin levels can be influenced by a host of factors unrelated to hepatic function, and albumin’s long half-life (15–20 days) makes it a poor marker for acute hepatic dysfunction. Most clotting factors are synthesized predominantly in the liver, except for factor VIII. Due to this fact the prothrombin time (PT) and international normalized ratio (INR) may also be used as markers of hepatic synthetic function. However, these too should be interpreted with caution as other condi­tions, including vitamin K deficiency and warfarin use, may prolong a patient’s PT/INR. (See Schwartz 11th ed., p. 1353.)
Answer: A
Imaging modalities have rapidly improved over the recent decades, and CT and MRI constitute the mainstays of diag­nostic imaging for patients with hepatic pathology. While routine B-mode and Doppler ultrasound represent excellent initial screening tests due to the fact that they are both widely accessible and inexpensive, most patients with pathology identified on ultrasound will require further imaging. When characterizing liver lesions by CT scan, contrast enhancement is necessary for a complete evaluation. Leveraging the dual blood supply of the liver with the hemodynamics of hepatic
tumors, radiologists are able to selectively enhance the liver
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parenchyma or tumor during the portal venous or arterial phases of the scan, respectively. Limitations of ultrasound include incomplete imaging of the liver, most often at the dome or beneath ribs on the surface, and incomplete visual­ization of lesion boundaries. Moreover, obesity and overlying bowel gas also can interfere with image quality. Thus, ultraso­nographically detected masses usually require further evalu­ation by other imaging modalities due to the lower sensitivity and specificity of ultrasound compared with CT and MRI. MRI offers advantages over CT in allowing higher soft tissue contrast and superior depiction of fluid-containing structures while eliminating the need for ionizing radiation. Develop­ment of specialized liver-specific contrast agents, including ferumoxide and gadoxetate, has led to the development of MRI that allows characterization of both hepatic structure and function. While positron emission tomography/com­puted tomography (PET/CT) is used frequently in the stag­ing and follow-up of patients with metastatic liver lesions, its role in the diagnosis and management of primary liver lesions is uncertain. The reported sensitivity of fluorodeoxyglucose (FDG)-PET/CT for the detection of hepatocellular carci­noma is only 50%–65%. While this has been improved with development of dual-tracer PET/CT, the clinical benefits of this modality have yet to be clearly defined. Despite advances in other imaging modalities, intraoperative liver ultrasound remains the gold standard. This technique has the ability to identify 20%–30% more lesions than preoperative imaging, and it is estimated that approximately 50% of planned sur­gical resections are influenced by information gleaned from intraoperative ultrasound. (See Schwartz 11th ed., p. 1357.)
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10. In a patient without prior liver disease, what is the most common cause of hepatic encephalopathy occurring within 26 weeks of severe liver injury? A. Alcohol in developing nations. B. Causes are unknown. C. Hepatitis B, A, and E in the United States. D. Drug-/toxin-induced (including acetaminophen) in
the United States.
Answer: D
Differences in etiology, management, and patient outcomes have been described for various regions of the globe. In the East and developing portions of the world, the most com­mon causes of acute liver failure (ALF) are viral infections, primarily hepatitis B, A, and E. In these areas, there are a rela­tively small number of drug-induced cases. In contrast, 65% of cases of ALF in the West are thought to be due to drugs and toxins, with acetaminophen (paracetamol) being the most common etiologic agent in the United States, Australia, United Kingdom, and most of Europe. In France and Spain, where acetaminophen sales are restricted, the rate of acet­aminophen-induced ALF is quite low. Acetaminophen­induced ALF is also uncommon in South America. The US Acute Liver Failure Study Group identified several other causes of ALF, including autoimmune hepatitis, hypoperfu­sion of the liver (in cardiomyopathy or cardiogenic shock), pregnancy-related conditions, and Wilson disease. Even with exhaustive efforts to identify a cause, approximately 20% of all cases of ALF remain indeterminate in origin. (See Schwartz 11th ed., p. 1361.)
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11. A patient presents with painless jaundice, and is found to have cirrhosis. They have no history of alcohol abuse, but do note a history of diabetes mellitus and pseudogout. They also mention that multiple members of their fam­ily have suffered from cirrhosis. What is the most likely etiology for their cirrhosis? A. Alcohol abuse B. Wilson disease
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C. α1-antitrypsin deficiency D. Hemochromatosis
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12. Which of the following clinical observations are associ­ated with the correct pathophysiologic explanations of cirrhosis? A. Spider angiomata, palmar erythema, caput medusae
due to portosystemic collateral formation in the set­ting of portal hypertension
B. Gynecomastia, loss of chest and axillary hair, and tes-
ticular atrophy from chronic spironolactone use
C. Abdominal wall hernias secondary to destruction of
type IV collagen
D. Caput medusae and the presence of the Cruveilhier-
Baumgarten murmur from collaterals between the portal system and the remnant of the umbilical vein
Answer: D
Chronic hepatitis C infection is the most common cause of chronic liver disease in the United States. Other etiologies include alcohol abuse, nonalcoholic steatohepaitis, and auto­immune diseases (primary biliary cirrhosis, primary scle­rosing cholangitis, and autoimmune hepatitis). Hereditary hemochromatosis is the most common metabolic cause of cirrhosis, and should be suspected if a patient presents with skin hyperpigmentation, diabetes mellitus, pseudogout, car­diomyopathy, or a family history of cirrhosis. Elevated plasma ferritin and increased iron saturation levels suggest the pres­ence of iron overload, but these findings also can be seen in other diseases of the liver. Confirmatory testing can be achieved by means of genetic testing, liver biopsy, or by assess­ing the response to phlebotomy. Other uncommon metabolic disorders leading to cirrhosis include Wilson disease and α1­antitrypsin deficiency. (See Schwartz 11th ed., p. 1364.)
Answer: D
The clinical history associated with cirrhosis can include fatigue, anorexia, weight loss, jaundice, abdominal pain, peripheral edema, ascites, gastrointestinal (GI) bleeding, and hepatic encephalopathy. On physical examination, a number of findings have been described in patients with cirrhosis. Spider angiomata and palmar erythema are believed to be caused by alterations in sex hormone metabolism. Finger club­bing may be a consequence of hypoalbuminemia, while the pathogenesis of white nail beds and Dupuytren contractures are less well understood. Males may develop features of femi­nization such as gynecomastia, loss of chest and axillary hair, and testicular atrophy. Splenomegaly is common, whereas the cirrhotic liver itself may be enlarged, normal sized, or small. Ascites and pleural effusion can be seen with fluid accumula­tion. Abdominal hernias are common with ascites and should be electively repaired only in patients with well-compensated cirrhosis; otherwise, the hernia should be repaired at the time of or after hepatic transplantation. Portal hyperten­sion can manifest as caput medusae and/or the presence of the Cruveilhier-Baumgarten murmur, a venous hum that can be auscultated in the epigastrium resulting from collaterals between the portal system and the remnant of the umbilical vein. (See Schwartz 11th ed., p. 1364.)
13. Clinically significant portal hypertension is evident when the _______ exceeds ______ mm Hg. A. wedged hepatic venous pressure; 10 B. free hepatic venous pressure; 20 C. hepatic venous pressure gradient; 10 D. hepatic venous pressure gradient; 20
Answer: C
Portal hypertension occurs when the pressure in the portal system is increased due to factors that may be divided into three categories. Presinusoidal causes of portal hyperten­sion include sinistral/extrahepatic (splenic vein thrombosis, splenomegaly, splenic AV fistula) and intrahepatic (schisto­somiasis, congenital hepatic fibrosis, idiopathic portal fibro­sis, myeloproliferative disorder, sarcoid, graft-versus-host disease) etiologies. Sinusoidal portal hypertension is a conse­quence of cirrhosis of any etiology. Postsinusoidal hyperten­sion can also be divided into intrahepatic (vascular occlusive disease) and posthepatic (Budd-Chiari, congestive heart failure [CHF], inferior vena cava [IVC] webs) etiologies. In evaluating patients with suspected portal hypertension, an enlarged portal vein on routine abdominal ultrasonography may suggest portal hypertension but this is not diagnostic.
Doppler ultrasound allows identification of vascular occlusion
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and the direction of portal venous flow. Computed tomogra­phy (CT) and magnetic resonance angiography (MRA) are useful for evaluating portal venous patency and anatomy. The most accurate method for measuring portal hyperten­sion is hepatic venography. This procedure introduces a bal­loon catheter directly into the hepatic vein where free hepatic venous pressure (FHVP) is measured. The hepatic vein is then occluded by inflation of the balloon allowing measurement of the wedged hepatic venous pressure (WHVP). The hepatic venous pressure gradient (HVPG) may then be calculated by subtracting the FHVP from the WHVP (HVPG = WHVP − FHVP). Clinically significant portal hypertension is defined as HVPG > 10 mm Hg. (See Schwartz 11th ed., p. 1365.)
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14. What is the leading cause of morbidity and mortality among patients with cirrhosis? A. Portal hypertension associated variceal bleeding B. Encephalopathy C. Development of hepatocellular carcinoma D. Hepatorenal syndrome
15. How should acute variceal hemorrhage be managed? A. Balanced blood product resuscitation supplemented
with recombinant factor VIIa B. Intravenous vasopressin and crystalloid infusion C. Emergent endoscopic evaluation and variceal
banding D. Trans jugular intrahepatic portosystemic shunting
(TIPS) followed by variceal banding
Answer: A
The most significant manifestation and the leading cause of morbidity and mortality related to portal hypertension is variceal bleeding. Approximately 30% of patients with com­pensated cirrhosis and 60% of patients with decompensated cirrhosis have esophageal varices. One-third of all patients with varices will experience variceal bleeding. Each episode of bleeding is associated with a 20% to 30% risk of mortality. If left untreated, 70% of patients who survive the initial bleed will experience recurrent variceal hemorrhage within 2 years of the index hemorrhage. (See Schwartz 11th ed., p. 1367.)
Answer: C
Patients with acute variceal hemorrhage should be admitted to an ICU for resuscitation and management. Blood resusci­tation should be performed carefully to reach a hemoglobin level of approximately 8 g/dL. Overzealous replacement of blood products and administration of saline can lead to both rebleeding and increased mortality. Administration of fresh frozen plasma and platelets can be considered in patients with severe coagulopathy. Use of recombinant factor VIIa has not been shown to be more beneficial than standard therapy and therefore is not recommended at this time.
Vasoactive medications decrease blood flow to the gastro­esophageal varices and can be initiated as soon as the diagno­sis of variceal bleeding is made. Although vasopressin is the most potent available vasoconstrictor, its use is limited by its systemic vasoconstrictive effects that can produce hyperten­sion, myocardial ischemia, arrhythmias, ischemic abdominal pain, and limb gangrene. Octreotide, a somatostatin analog, has the advantage that it can be administered for 5 days or longer, and it is currently the preferred pharmacologic agent for initial management of acute variceal bleeding. In addi­tion to pharmacologic therapy, endoscopy with variceal band ligation should be carried out as soon as possible. This com­bination of pharmacologic and endoscopic therapy has been shown both to improve the initial control of bleeding and to increase the 5-day hemostasis rate. TIPS can be performed in 95% of patients by an experienced interventional radiolo­gist, controls variceal bleeding in >90% of cases refractory to medical treatment, and should not affect subsequent hepatic transplantation. (See Schwartz 11th ed., p 1367.)
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16. A 52-year-old man with no travel history, chronic pul­monary obstructive disease, and recurrent diverticulitis presents with increasing right upper quadrant abdomi­nal pain, fevers, and jaundice. Computed tomography (CT) imaging notes a single, 5 cm hypodense lesion with peripheral enhancement in the right lobe of the liver. What is the best treatment for this patient? A. Metronidazole and percutaneous drainage
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B. Metronidazole C. Mebendazole D. Intravenous piperacillin/tazobactam and percutane-
ous drainage
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Answer: D
Pyogenic liver abscesses are the most common liver abscesses seen in the United States. Patients commonly present with right upper quadrant pain and fever. Jaundice occurs in up to one-third of affected patients. They may be single or multiple and are more frequently found in the right lobe of the liver. The abscess cavities are variable in size and, when multiple, may coalesce to give a honeycomb appearance. Approximately 40% of abscesses are monomicrobial, an additional 40% are polymicrobial, and 20% are culture-negative. The most com­mon infecting agents are gram-negative bacteria. Escherichia coli is found in two-thirds of cases, and other common organ­isms include Streptococcus faecalis, Klebsiella, and Proteus vulgaris. Anaerobic organisms such as Bacteroides fragilis also are seen frequently. In patients with endocarditis and infected indwelling catheters, Staphylococcus and Streptococcus species are more commonly found. Ultrasound examination of the liver reveals pyogenic abscesses as round or oval hypoechoic lesions with well-defined borders and a variable number of internal echoes. CT scan is highly sensitive in the localiza­tion of pyogenic liver abscesses, which appear hypodense with peripheral enhancement and may contain air-fluid levels indicating a gas-producing infectious organism.
The current cornerstones of treatment include correction of the underlying cause and intravenous (IV) antibiotic ther­apy. Empiric antibiotic therapy should cover gram-negative and anaerobic organisms; percutaneous needle aspiration and culture of the aspirate may be useful in guiding subsequent antibiotic therapy. IV antibiotic therapy should be contin­ued for at least 8 weeks and can be expected to be effective in 80% to 90% of patients. Placement of a percutaneous drain­age catheter is beneficial only for a minority of patients, as most pyogenic abscesses are quite viscous and catheter drain­age is often ineffective (Fig. 31-8). (See Schwartz 11th ed., Figure 31-16, p. 1369.)
FIG. 31-8. Computed tomographic scan of pyogenic liver
abscesses. Multiple hepatic abscesses are seen in a patient after an episode of diverticulitis. Note the loculated large central abscess as well as the left lateral segment abscess.
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17. Upon return from New Zealand, a 33-year-old woman presents with dull right upper quadrant pain and fevers diagnosed by the presence of echinococcal antigens. What does the computed tomography (CT) imaging demonstrate. A. Multiple, well circumscribed lesions with homog-
enous arterial enhancement and central scar
B. Well-defined low-density round lesions that have
enhancement of the wall, somewhat ragged in appearance with a peripheral zone of edema in the left lobe
C. Anteroinferior hypodense lesion with a distinct wall
and ring-like calcifications in the right lobe
D. Asymmetrical nodular peripheral enhancement that
is isodense with large vessels and exhibit progressive centripetal enhancement fill-in over time in the right lobe
Answer: C
Hydatid disease is most common in sheep-raising areas, where dogs have access to infected offal. These include South Australia, New Zealand, Africa, Greece, Spain, and the Mid­dle East. Hydatid cysts commonly involve the right lobe of the liver, usually the anterior-inferior or posterior-inferior segments. Occasionally, the affected patient presents with symptoms such as dull right upper quadrant pain or abdomi­nal distention.
The diagnosis of hydatid disease is based on the findings of an enzyme-linked immunosorbent assay (ELISA) for echino­coccal antigens, and results are positive in approximately 85% of infected patients. Ultrasonography and CT scanning of the abdomen are both quite sensitive for detecting hydatid cysts. The appearance of the cysts on images depends on the stage of cyst development. Typically, hydatid cysts are well-defined hypodense lesions with a distinct wall. Ring-like calcifica­tions of the pericysts are present in 20% to 30% of cases. As healing occurs, the entire cyst calcifies densely, and a lesion with this appearance is usually dead or inactive. Daughter cysts generally occur in a peripheral location within the main cyst and are typically slightly hypodense compared with the mother cyst. Magnetic resonance imaging (MRI) of the abdo­men may be useful to evaluate the pericyst, cyst matrix, and daughter cyst characteristics. (See Schwartz 11th ed., p. 1370.)
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18. What is the most common complication following tran­sjugular intrahepatic portosystemic shunt (TIPS)? A. Encephalopathy B. Hepatic ischemia C. Infection D. Life-threatening hemorrhage
Answer: A
TIPS is a percutaneous procedure used for treatment of patients who have gastroesophageal varices in the setting of portal hypertension. It has largely replaced surgical portosys­temic shunts due to the fact that it is both safe and effective while also providing a minimally invasive alternative to major abdominal surgery. TIPS functions by creating an intrahe­patic shunt between the portal and systemic circulation which causes a reduction in the portal pressure and ultimately in the blood flow through varices. It is accomplished by endovascu­lar access through the jugular vein to a hepatic vein radical and subsequent creation of a needle tract that connects it to a branch of the portal vein. After dilation of the tract, a metallic stent is deployed to hold the new portosystemic connection open. Because this shunt reduces first pass metabolism of the liver, the most common complication of TIPS is encephalopa­thy which occurs in 25%–30% of patients. Other complica­tions such as hepatic ischemia, infection, renal failure and hemorrhage may occur, but are rare. (See Schwartz 11th ed., p. 1367.)
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19. A patient presents to your clinic for follow up of his Hepatitis C cirrhosis with mild ascites, an albumin of
2.9 g/dL, creatinine of 2.1 mg/dL, sodium of 121 mEq/L, international normalized ratio (INR) of 1.6, and bili­rubin of 1.9 mg/dL and complete resolution of prior hepatic encephalopathy. A. Based upon his clinical presentation and labora-
tory findings, his Model for End-Stage Liver disease
CHAPTER 31
(MELD) score is 26.
B. Summation of his creatinine, INR, and bilirubin indi-
cates he has a Child-Pugh Score in Class B.
C. Based upon clinical and laboratory values, he has a
Child-Pugh Score in Class A.
D. Formulation of his laboratory values indicate his
MELD is 29.
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Answer: D
The MELD is a linear regression model based on three objec­tive laboratory values (INR, bilirubin level, and creatinine level). It was originally developed as a tool to predict mortal­ity after transjugular intrahepatic portosystemic shunt (TIPS) but has been validated and used as the sole method of liver transplant allocation in the United States since 2002. The MELD formula accounts for objective valuation creatinine, INR, bilirubin, creatinine with serum sodium subsequently added to improve sensitivity and specificity.
The Child-Turcotte-Pugh (CTP) score was originally developed to evaluate the risk of portocaval shunt procedures performed for portal hypertension and subsequently has been shown to be useful in predicting surgical risks of other intra-abdominal operations on cirrhotic. Numerous studies have demonstrated overall surgical mortality rates of 10% for patients with class A cirrhosis, 30% for those with class B cirrhosis, and 75% to 80% for those with class C cirrhosis. Ranging from 5 to 15, the CTP is calculated based upon a summation of points derived from the presence of ascites and encephalopathy, serum bilirubin and albumin levels, as well as INR. Multiple retrospective studies have demonstrated that perioperative mortality and morbidity rates correlate well with the CTP score, and for over 30 years, this measure had been used as the principal predictor of operative risk (Table 31-1). (See Schwartz 11th ed., Table 31-4, p. 1365.)
TABLE 31-1 Child-Turcotte-Pugh (CTP) score
Variable 1 Point 2 Points 3 Points
Bilirubin level < 2 mg/dL 2–3 mg/dL > 3 mg/dL
Albumin level > 3.5 g/dL 2.8–3.5 g/dL < 2.8 g/dL
International normalized ratio < 1.7 1.7–2.2 > 2.2
Encephalopathy None Controlled Uncontrolled
Ascites None Controlled Uncontrolled
Child-Turcotte-Pugh class
Class A = 5–6 points Class B = 7–9 points Class C = 10–15 points
20. The most common benign hepatic lesion is the: A. Hemangioma. B. Simple cyst. C. Adenoma. D. Bile duct hamartoma.
Answer : B
While hemangiomas are the most common solid benign masses found in the liver, the simple hepatic cyst is still the most common overall. Simple cysts have a prevalence of approximately 2.8%–3.6%, and are more common in women by a ratio of 4:1. Cysts are generally found incidentally dur­ing abdominal imaging, and small, asymptomatic cysts may be managed conservatively. Large cysts may begin to cause abdominal pain, epigastric fullness and early satiety. These patients may be treated with percutaneous cyst aspiration and sclerotherapy which is effective in approximately 90% of patients. For those that fail percutaneous treatment, or where percutaneous treatment is not available, surgical cyst fenestration may be considered. If surgical fenestration is per­formed, the cyst wall should be sent for pathologic analysis to exclude carcinoma. (See Schwartz 11th ed., p. 1373.)
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21. Which of the following liver lesions carry a significant risk of spontaneous rupture? A. Hemangioma B. Hepatic cyst C. Adenoma D. Bile duct hamartoma
22. A patient presents with results from a contrast-enhanced computed tomography (CT) scan that describe a well­circumscribed lesion that demonstrates homogenous enhancement during arterial phase, isodensity on the venous phase, and a central scar. In general, what would be the recommended treatment? A. Reassurance and observation B. Percutaneous radio frequency ablation C. Resection D. Transarterial chemoembolization
Answer: C
Hemangiomas are congenital vascular lesions that may range in size from <1 cm to ≥25 cm. They are predominantly found in women, and are generally asymptomatic. Large lesions may result in discomfort from compression of nearby organs. Though hemangiomas are at risk for bleeding if they are biop­sied, spontaneous rupture is rare. Adenomas, on the other hand, carry a significant risk for spontaneous rupture with intraperitoneal bleeding. For this reason, along with their potential for malignant degeneration, it is generally recom­mended that hepatic adenomas be resected once discovered. (See Schwartz 11th ed., p. 1375.)
Answer: A
On contrast-enhanced imaging, an focal nodular hyperpla­sia (FNH) can be recognized as a well-circumscribed mass that demonstrates enhancement on the arterial phase and isointensity on the venous phase. FNH also demonstrates a characteristic central scar. FNH are solid benign lesions are similar to adenomas in that they are more common in women of childbearing age. Unlike adenomas, however, they are not prone to malignant degeneration or spontaneous rupture. For this reason, asymptomatic FNHs may be managed conserva­tively unless adenoma or hepatocellular carcinoma (HCC) cannot be definitively excluded. Gadolinium-enhanced mag­netic resonance imaging (MRI) may allow better visualization of the fibrous septa extending from the FNH’s central scar. While FNH and adenomas ma appear similar on computed tomography (CT) or standard MRI, new MRI contrast agents like gadobenate dimeglumine (MultiHance) allow supe­rior discrimination between these two lesions (Fig. 31-9). (See Schwartz 11th, Fig. 31-18, p. 1375.)
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Liver
FIG. 31-9. Computed
tomographic scans showing classic appearance of benign liver lesions. Focal nodular hyperplasia (FNH) is hypervascular on arterial phase, isodense to liver on venous phase, and has a central scar (upper panels). Adenoma is hypovascular (lower left panel). Hemangioma shows asymmetrical peripheral enhancement (lower right panel).
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23. What is the annual conversion rate to hepatocellular carcinoma (HCC) for patients with cirrhosis? A. Less than 1% B. 1%–2% C. 2%–6% D. 6%–10%
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24. Which of the following is TRUE in regard to patients with cholangiocarcinoma? A. Surgical resection is the treatment of choice for
patients with primary sclerosing cholangitis and cholangiocarcinoma limited to the common bile duct.
B. Cholangiocarcinoma is very radiosensitive, there-
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fore external-beam radiation and intravenous 5- fluorouracil constitute destination therapy with 60% 5-year survival.
C. 5-year survival rate following the Mayo Clinic proto-
col and transplantation is 70%.
D. Patients with primary sclerosing cholangitis are not
candidates for the Mayo Clinic protocol.
Answer: C
HCC is the fifth most common malignancy worldwide, and its risk factors include viral hepatitis, alcoholic cirrhosis, hemochromatosis, and NASH. Cirrhosis is present in 70% to 90% of patients who develop HCC, and the annual conver­sion rate from cirrhosis is 2% to 6%. (See Schwartz 11th ed., p. 1376.)
Answer: C
Cholangiocarcinoma, or bile duct cancer, is the second most common primary malignancy of the liver. Cholangiocarci­noma is an adenocarcinoma of the bile ducts; it forms in the biliary epithelial cells and can be subclassified into peripheral (intrahepatic) bile duct cancer and central (extrahepatic) bile duct cancer. Extrahepatic bile duct cancer can be located dis­tally or proximally. When proximal, it is referred to as a hilar cholangiocarcinoma (Klatskin’s tumor).
In the absence of associated primary sclerosing cholan­gitis (PSC), surgical resection is the treatment of choice for hilar cholangiocarcinoma. However, approximately 10% of patients with cholangiocarcinoma have PSC. Furthermore, cholangiocarcinoma in the setting of PSC is frequently mul­ticentric and often is associated with underlying liver disease, with eventual cirrhosis and portal hypertension. As a result, experience has shown that resection of cholangiocarcinoma in patients with PSC yields dismal results.
The pretransplant Mayo protocol consists of external­beam radiation therapy plus a protracted course of intrave­nous 5-fluorouracil followed by iridium-192 brachytherapy. Patients then undergo an abdominal exploration with stag­ing. If findings are negative, patients are given capecitabine for 2 of every 3 weeks until transplantation. Even after restag­ing with CT/MRI and endoscopic ultrasonography, approxi­mately 15% to 20% of patients will have positive findings for tumor on abdominal exploration. The 5-year survival rate for those undergoing transplantation for cholangiocarcinoma at the Mayo Clinic is approximately 70% and compares favor­ably with the rate for resection. Current eligibility criteria for this Mayo Clinic protocol include unresectable hilar cholan­giocarcinoma or hilar cholangiocarcinoma with PSC. The tumor must have a radial dimension of ≤3 cm with no intra­hepatic or extrahepatic metastases, and the patient must not have undergone prior radiation therapy or transperitoneal biopsy. Many centers have adopted similar protocols with comparable results. (See Schwartz 11th ed., p. 1377.)
25. A patient undergoes routine cholecystectomy and is incidentally found to have gallbladder carcinoma with­out invasion into the muscular layer. Further treatment should include: A. No further treatment. B. External beam radiation with systemic chemotherapy. C. Reoperation with a segment IVb/V resection and
hilar lymphadenectomy.
D. Reoperation with formal lobectomy and bile duct
resection.
Answer: A
Gallbladder cancer is a rare and aggressive form of biliary malignancy. In approximately one-third of cases it is diag­nosed incidentally following routine cholecystectomy. Treat­ment for these patients is guided by T stage of the tumor. In those patients with T1a tumors, as in this question, no further treatment is necessary. If the tumor invades into the muscular layer (T1b), reoperation with central liver resection and hilar lymphadenectomy is recommended. The role for more radi­cal resections is unclear. (See Schwartz 11th ed., p. 1378).
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26. Which of the following is considered a primary determi­nant of suitability for resection when evaluating a patient with hepatic colorectal metastases? A. Number of metastatic tumors B. Size of metastatic tumors C. Predicted volume of hepatic remnant D. Prior therapy
27. Based on the standard Milan criteria, which of the fol­lowing patients with hepatocellular carcinoma (HCC) would be eligible for transplantation? A. One 4.5 cm lesion in segment VI with invasion of the
right portal vein
B. Three lesions confined to the right lobe, with the
largest being 2.5 cm C. A single, 5.5 cm lesion in segment II D. Three lesions spread throughout the liver, with the
largest being 3.5 cm
Answer: C
The liver is a common site for metastatic disease in patients with colorectal disease, and approximately 50% to 60% of patients diagnosed with colorectal cancer will develop liver metastases within their lifetime. With the advent of more aggressive strategies for the management of metastatic colorec­tal cancer, including improved chemotherapeutic regimens and expanded use of metastasectomy, the 5-year survival for patients with isolated metastases to the liver may exceed 30%. Given these encouraging results, the paradigm for surgical evaluation and treatment of these patients has shifted to pri­marily consider the health of the background liver and volume of the hepatic remnant, and not tumor characteristics like size and number. (See Schwartz 11th ed., p. 1378.)
Answer: B
Orthotopic liver transplantation (OTL) was first attempted in the 1980s and 1990s, with initial series reporting 5-year sur­vival rates of 20% to 50%. This led to the introduction of the Milan criteria which limited eligibility to patients with one tumor <5 cm or up to three tumors <3 cm and no evidence of gross intravascular or extrahepatic spread. Adoption of these guidelines resulted in significant improvement in 5-year sur­vival for patients with HCC treated with OTL. (See Schwartz 11th ed., p. 1380.)
CHAPTER 31
Liver
28. The only FDA-approved systemic chemotherapeutic agent for hepatocellular carcinoma (HCC) is: A. Epirubicin. B. Cisplatin. C. 5-fluorouracil. D. Sorafenib.
Answer: D
Though systemic chemotherapy has not proven very effective in the treatment of HCC, the multikinase inhibitor sorafenib has been approved for use specifically in these patients. Based on results of the SHARP trial, there sorafenib demonstrated a 3-month survival benefit versus placebo. Though these results are modest, it remains a treatment option for patients with advanced, unresectable HCC. (See Schwartz 11th ed., p. 1381.)