17 Pancreatic and Hepatobiliary Cancer

Thomas Regenbogen and Andrea Wang-Gillam

QUESTIONS

Each of the numbered items below is followed by lettered answers. Select the ONE lettered answer that is BEST in each case unless instructed otherwise.

Question 17.1 Which of the following factors are associated with an increased risk of pancreatic cancer?

A. Cigarette smoke

B. Chronic pancreatitis

C. Prior cholecystectomy

D. African-American ethnicity

E. All of the above

Question 17.2 Which of the following statements regarding pancreatic cancer is TRUE?

A. At diagnosis, 31% have evidence of distant metastases.

B. Pancreatic cancer decreases in incidence later in life.

C. Activation of the KRAS oncogene plus inactivation of tumor suppressor genes (TP53, DPC4, p16, and BRCA2) are associated with the development of pancreatic cancer.

D. Pancreatic intraepithelial neoplasms (PanINs) are intraductal proliferative epithelial lesions that will not progress to pancreatic cancer.

E. Patients with advanced pancreatic cancer will have high levels of CA 19-9 if they are Lewis antigen-a or -b negative.

Question 17.3 A 56-year-old man is evaluated for a 1-month history of gradually worsening painless jaundice and a 10-lb weight loss. Computed tomography (CT) scan of the abdomen and pelvis revealed a 3.3-cm pancreatic head mass adjacent to the superior mesenteric vein with no intervening fat plane and encasing both the superior mesenteric vein and artery. The common bile duct was dilated and the pancreatic body and tail were atrophied with dilatation of the pancreatic duct. Portal lymphadenopathy measuring 2 cm was present. The patient underwent endoscopic retrograde cholangiopancreatography and a biliary stent was placed. Biopsy tissue of the mass was consistent with moderately differentiated pancreatic adenocarcinoma. What stage is this patient’s cancer per the TNM staging system?

A. Stage I

B. Stage II

C. Stage III

D. Stage IV

Question 17.4 A 67-year-old woman presents to her local emergency department with a 2-month history of right upper quadrant pain, jaundice, and 20-lb weight loss. CT of the abdomen with contrast reveals a 2.5-cm, ill-defined soft tissue density within the head of the pancreas and mild celiac axis, porta hepatis, and portacaval adenopathy, with the largest being within the celiac axis region measuring 2.0 × 1.8 cm. Following admission endoscopic retrograde cholangiopancreatography with biliary stent placement and biopsy is done. Pathology reports poorly differentiated adenocarcinoma. What test should be ordered at this point to help establish her stage?

A. Multiphase multidetector helical computerized tomography

B. Magnetic resonance imaging

C. Ultrasonography

D. Serum CA 19-9 measurement

E. Endoscopic ultrasonography

Question 17.5 A 71-year-old woman undergoes Whipple resection for a T3N1M0 pancreatic adenocarcinoma. Postoperative recovery was uneventful, and she starts adjuvant therapy 7 weeks later with gemcitabine given intravenously weekly for 3 weeks, followed by a 1-week break. In a follow-up visit after her first cycle, she reports a 5-lb weight loss, nausea, decreased appetite, and diarrhea with floating stools. What should be done next?

A. Increase pancreatic enzyme supplementation

B. Admit the patient for small bowel obstruction

C. Hold chemotherapy for 1 week and follow up on symptoms

D. CT scan of the chest, abdomen, and pelvis to rule out metastatic disease

Question 17.6 Which of the following statements regarding adjuvant therapy of pancreatic adenocarcinoma is TRUE?

A. In RTOG 9704 administering gemcitabine before and after adjuvant 5-FU based CRT for resected pancreatic head adenocarcinoma demonstrated a trend toward improved median OS compared with 5-FU before and after 5-FU CRT.

B. CONKO-001 demonstrated gemcitabine when compared with observation improves disease free survival but not overall survival.

C. GITSG 9173 clearly showed no benefit with 5-FU based chemoradiation followed by chemotherapy compared with observation.

D. ESPAC-1 showed that those who received CRT did better than those treated with chemotherapy alone.

Question 17.7 A 64-year-old woman is diagnosed with locally advanced unresectable pancreatic cancer. After 2 months of a gemcitabine-based chemotherapy regimen, CT scans demonstrate a decrease in the size of the lesion and her CA19-9 decreased from 854 to 201. What should be done now?

A. Switch to 5FU-based chemoradiation

B. Continue current gemcitabine-based chemotherapy for another 2 months

C. Add radiation to her current regimen

D. Add 5FU to her chemotherapy regimen

Question 17.8 A 52-year-old woman is evaluated for chronic right upper quadrant abdominal pain and is diagnosed with pancreatic adenocarcinoma with metastasis to the liver. She is distraught and wants to do everything possible to prolong her life. Which of the following regimens has been shown to prolong overall survival compared with gemcitabine alone?

A. Gemcitabine and oxaliplatin

B. Gemcitabine and capecitabine

C. Gemcitabine and nab-paclitaxel

D. Gemcitabine, cisplatin, and bevacizumab

E. Gemcitabine and cetuximab

Question 17.9 A 45-year-old man with unintentional weight loss is diagnosed with metastatic adenocarcinoma of the pancreas. Which of the following chemotherapy regimens would be appropriate first-line treatment choice in this otherwise healthy patient with normal organ function?

A. Gemcitabine

B. FOLFIRINOX (5FU, leucovorin, irinotecan, and oxaliplatin)

C. 5FU with radiation

D. FOLFOX (5FU + leucovorin + oxaliplatin)

Question 17.10 Mutation of which tumor suppressor gene is most frequently associated with familial pancreatic cancer?

A. BRCA2

B. PALB2

C. KRAS

D. TP53

Question 17.11 Which one of the following statements regarding risk factors for pancreatic cancer is CORRECT?

A. People with blood types A, B, and AB are more likely than type O to develop pancreatic cancer.

B. Testing for KRAS mutations in the pancreatic juice of patients is an effective screening test for pancreatic cancer.

C. Patients with hereditary nonpolyposis colorectal cancer syndrome do not have an increased risk for pancreatic cancer.

D. Hereditary pancreatitis is not a significant risk factor for pancreatic cancer.

Question 17.12 A 65-year-old male diagnosed with metastatic pancreatic cancer received treatment with single-agent gemcitabine. He now has disease progression and is interested in pursuing further systemic therapy. Which one of the following should be offered as a second-line treatment choice?

A. OFF (oxaliplatin, 5FU, and leucovorin)

B. Erlotinib

C. Paclitaxel

D. Supportive care alone

Question 17.13 Which of the following is/are TRUE about pancreatic cancer?

A. Most pancreatic cancers have mutations in KRAS, TP53, SMAD4, p16/CDKN2A.

B. Telomere shortening is the earliest and prevalent genetic change identified in the precursor lesions.

C. Underexpression of TGF-β is observed in some pancreatic cancers.

D. p16-mediated CDK inhibition is a protective mechanism against pancreatic cancer.

E. All of the above.

Question 17.14 A 52-year-old man is found to have mildly abnormal liver function and an elevated serum α-fetoprotein (AFP). Workup reveals prior hepatitis B viral (HBV) infection. Ultrasound reveals a 4-cm lesion in the left hepatic lobe, and a computed tomography (CT) scan reveals no evidence of metastatic disease or vascular involvement. MRI demonstrates features consistent with hepatocellular carcinoma (HCC). The patient undergoes partial hepatectomy, surgical margins are clear. The pathology report confirms the diagnosis of HCC. Which of the following approaches should be followed?

A. Adjuvant sorafenib

B. Combination chemotherapy that is doxorubicin based

C. Adjuvant external beam radiation to surgical bed

D. Routine surveillance

Question 17.15 Which of the following criteria help(s) guide selection of patients with HCC appropriate for potential liver transplantation?

A. Patients with solitary tumors ≤5 cm, or patients with multifocal disease with ≤3 tumor nodules each ≤3 cm in size

B. Patients with Child–Pugh B or C cirrhosis

C. Tumors without evidence of macrovascular invasion and distant metastasis

D. All of the above

Question 17.16 Which of the following statements about staging systems for hepatocellular carcinoma is TRUE?

A. The Okuda system takes into account several clinical features that include tumor size (>50% of liver), ascites (positive or negative), hypoalbuminemia (<3 g/dL), and hyperbilirubinemia (>3 mg/dL).

B. The Cancer of the Liver Italian Program system uses hepatic tumor morphology and extent of liver replacement, Child–Pugh score, portal vein thrombosis, and serum AFP levels.

C. The Barcelona Clinic Liver Cancer scoring system combines assessment of tumor stage, liver function, and patient symptoms with a treatment algorithm and has been shown to correlate well with patient outcomes.

D. All of the above.

Question 17.17 Which of the following abnormalities is both a paraneoplastic syndrome associated with HCC, and may also be caused by end-stage liver failure?

A. Hypoglycemia

B. Erythrocytosis

C. Hypercalcemia

D. Hypercholesterolemia

Question 17.18 An increased risk of developing HCC is associated with which of the following?

A. Wilson disease

B. Hereditary tyrosinemia

C. Porphyria cutanea tarda

D. Primary biliary cirrhosis

E. All of the above

Question 17.19 Which of the following statements about screening and prevention is CORRECT?

A. The advent of vaccination for hepatitis B is unlikely to reduce HCC in endemic areas.

B. A combination of AFP and ultrasound screening is used in high-risk populations.

C. Aggressive screening programs for HCC have been shown to improve survival.

D. Detection of HCC, through surveillance of patients awaiting liver transplantation, does not increase priority for orthotopic liver transplantation.

Question 17.20 Which of the following increase(s) the risk of developing cholangiocarcinoma?

A. Primary sclerosing cholangitis

B. Clonorchis sinensis infestation

C. Chronic portal bacteremia and portal phlebitis

D. All of the above

Question 17.21 A 66-year-old man is noted to have painless jaundice on a routine follow-up at his primary care physician’s office. Workup reveals a mass causing biliary obstruction at the hilum. Endoscopic retrograde cholangiopancreatography confirms a high-grade stricture predominantly involving the left hepatic duct; however, brushings reveal atypical cells and no malignancy. He is seen at a tertiary care center and offered surgical management, an en bloc resection of the left hepatic lobe and extrahepatic bile duct, and a complete periportal lymphadenectomy. Which of the following statements about management/natural history of hilar cholangiocarcinoma is/are TRUE?

A. Surgical resection is associated with an operative mortality rate of 30%.

B. Recurrences occur most commonly at the bed of resection, followed by retroperitoneal lymph nodes. Distant metastases occur in one-third of cases.

C. Less than 10% of patients have resectable cancer at the time of diagnosis.

D. All of the above.

Question 17.22 A 70-year-old man presents with 16-lb weight loss and persistent right upper quadrant pain. CT scan reveals a gall bladder stone and thickening of the anterior wall of the gall bladder. He undergoes a laparoscopic cholecystectomy. Pathology reveals a moderately differentiated 2-cm gallbladder adenocarcinoma invading the perimuscular connective tissue. Margins of resection are negative for tumor. Based on the available information, what is the stage of this cancer?

A. Stage IA

B. Stage IB

C. Stage II

D. Stage IIIA

Question 17.23 The patient in Question 17.22 recovers from surgery and seeks a second opinion at a tertiary care center 5 weeks after his cholecystectomy. A CT scan 2 weeks after surgery shows mild periportal fullness. What is the most appropriate next step in management?

A. Perform en bloc resection of the gallbladder, resection of segments IVb and V of the liver, and regional lymph node dissection.

B. No further therapy is warranted; surveillance with CT scans and laboratories done every 3 months.

C. He requires a second laparotomy to assess the extent of remaining disease to guide further therapy.

D. Perform ultrasound-guided biopsy of the periportal nodes; if positive, then fluoropyrimidine-based chemoradiation is indicated.

Question 17.24 Which one of the following statements about adjuvant therapy for biliary cancers is TRUE?

A. Based on retrospective data it appears patients may benefit from adjuvant chemotherapy.

B. Adjuvant radiation is superior to chemotherapy alone.

C. Adjuvant therapy can improve overall survival for patients with R0 resections.

D. Fluoropyrimidine-based chemoradiation is standard because it is superior to radiation alone.

Question 17.25 Which of the following statements regarding fibrolamellar HCC is CORRECT?

A. Fibrolamellar HCC occurs more frequently in men compared with women.

B. This variant of HCC is associated with viral hepatitis but not cirrhosis.

C. Lymph node metastases at the time of presentation is common.

D. Most patients with fibrolamellar HCC present in their sixth decade of life.

Question 17.26 A 50-year-old man with a history of chronic hepatitis B infection and Child–Pugh A cirrhosis presents with abdominal pain. He is otherwise in good health. CT of the abdomen reveals a cirrhotic liver with a 2.5-cm liver mass that rapidly enhances during the arterial phase of contrast administration and “washout” during the later venous phases. There is no involvement of the portal vein. AFP is 300 ng/mL. What is the next best step in his management?

A. Biopsy to obtain a histologic diagnosis.

B. Refer him to a hepatobiliary surgeon.

C. Start him on sorafenib.

D. Refer him to a radiation oncologist.

Question 17.27 Which of the following statements is CORRECT regarding hepatoblastoma?

A. This is the most common primary cancer of the liver in adults.

B. Hepatoblastoma is a chemoresistant tumor.

C. Patients with this tumor have a poor outcome after liver transplantation, with a 5-year survival rate of 20%.

D. The peak incidence of hepatoblastoma is within the first 2 years of life.

Question 17.28 Which of the following statements is CORRECT?

A. The hepatitis B x gene product has no known role in causing HCC.

B. NS5A protein product of hepatitis C viral (HCV) genome inactivates p53.

C. Level of HBV replication is inversely related to the risk of liver cancer.

D. Hepatitis B virus genotype C is associated with decreased risk of HCC.

Question 17.29 Which of the following statement(s) regarding hepatitis C infection is/are CORRECT?

A. Sixty percent to 80% HCV infections become chronic in contrast to 10% HBV infections.

B. HBV genome integrates into hepatocyte DNA while HCV genome does not.

C. The average interval from HCV infection to HCC is 30 years in contrast to 40 to 50 years for HBV infection.

D. All of the above.

Question 17.30 A 45-year-old man with a history of alcoholic cirrhosis was found by screening ultrasound to have two new lesions in hepatic segments 7 and 3, measuring 3 cm and 2 cm, respectively. MRI findings were consistent with hepatocellular carcinoma. He has been abstinent from alcohol use for the past 2 years. His total bilirubin is 1.5 mg/dL, serum albumin 3 g/dL and INR is 1.6. He does not have ascites or encephalopathy. There is no evidence of metastatic disease and he is referred for orthotopic liver transplantation evaluation. He is deemed to be an acceptable candidate. While waiting for a donor organ, what should be done next?

A. Refer to interventional radiology for ablative therapy

B. Sorafenib to prevent disease progression while waiting for donor organ

C. Cisplatin, IFNα-2b, doxorubicin, and 5FU as neoadjuvant therapy

D. Observation

Question 17.31 A 45-year-old woman presents with painless jaundice and weight loss. Her total bilirubin is 17.6 mg/dL, alkaline phosphatase is 568 units/L and alanine transaminase (ALT) is 138 units/L. Viral hepatitis serology is negative. CT chest abdomen and pelvis with contrast is performed and reveals an ill-defined, infiltrative, hypoattenuating lesion at the hepatic hilum with marked intrahepatic biliary ductal dilatation. MRI of the liver characterizes the lesion as compatible with cholangiocarcinoma centered in the hepatic hilum extending to the confluence of the left medial and lateral hepatic ducts, involving the cystic duct, proximal extrahepatic duct, and abutting the undersurface of the portal vein and right hepatic artery. ERCP is performed with common bile duct stenting. Pathology reports atypical epithelial cells in the brushing. Hepatobiliary surgery is consulted and determines that her tumor is unresectable and she is not a liver transplant candidate due to psychosocial issues. What is the next step in management once hyperbilirubinemia resolves?

A. FOLFOX

B. Gemcitabine and cisplatin

C. External beam radiation therapy with concurrent 5FU

D. Palliative care alone including routine stent exchange

Question 17.32 Which of the following individuals would meet criteria to have surveillance for HCC?

A. A 28-year-old African woman who is a hepatitis B carrier.

B. A 47-year-old Indian woman who is hepatitis B carrier and has a brother with HCC.

C. A 62-year-old Hispanic male with alcoholic cirrhosis.

D. A 42-year-old Chinese man who is a hepatitis B carrier.

E. All of the above

Question 17.33 A 52-year-old man is admitted to the hospital due to new onset of symptomatic ascites and jaundice. He is an alcoholic and has no history of medical care prior to this encounter. He is mildly encephalopathic. Serum total bilirubin is 5.6 mg/dL and INR is 2.1. He is diagnosed with unresectable metastatic hepatocellular carcinoma and alcoholic cirrhosis after an extensive work up. The patient’s family arrives and would like to discuss treatment options. Your recommendation will be:

A. Gemcitabine and oxaliplatin.

B. Sorafenib.

C. Sunitinib.

D. Hospice or supportive care alone

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