Practice question answers, exocrine pancreas/gallbladder

Practice question answers

Exocrine pancreas/gallbladder

© Jun Wang, MD, PhD

1. D. Any patient with acute onset of epigastric pain should be considered for acute pancreatitis. The two most commonly used lab tests are amylase and lipase. Bilirubin levels are used to determine possible causes of jaundice, not for pancreatitis. CA19-9 may be increased in mucinous neoplasms, including pancreatic adenocarcinoma and ovarian mucinous neoplasms. Culture is for bacterial infection. Elevation of troponin can be seen in myocardial injury, and other non-cardiac conditions, such as sepsis, chronic kidney disease, etc.

2. A. Diagnosis of acute pancreatitis requires two of three criteria, acute abdominal pain, lipase or amylase levels three times or more above the upper limits and/or radiologic findings of signs of inflammation, such enlargement and exudates. Barium swallow can be used to detect esophagus abnormalities, such tumors, or achalasia. D-dimer is used to detect coagulation disorders, such as DIC, that can be caused by acute pancreatitis. Mesentery angiography is used to diagnose mesentery thrombosis. Upper endoscopy can be used to exam esophageal and stomach abnormalities.

3. A. The image reveals enlarged of pancreas and peripancreatic exudates, consistent with acute pancreatitis. Chronic pancreatitis usually presents with triad of steatorrhea, diabetes and pancreatic calcifications. Mucinous cystic neoplasm, pancreatic adenocarcinoma and serous cystadenoma all have identifiable mass, not generalized pancreas enlargement and exudate. All these four have longer history instead of sudden onset.

4. C. Acute pancreatitis is associated with various risk factors, including hyperlipidemia, as seen in the history of this patient. Although alcohol is a leading cause of both acute pancreatitis and chronic pancreatitis, this patient does not have any history of alcohol usage. Helicobacter is associated with gastritis and peptic ulcer, but not pancreatitis. Scorpion bite may be associated with acute pancreatitis in Trinidad. Trypsinogen 1 mutation is seen in hereditary pancreatitis.

5. A. Hemorrhage and necrosis of pancreas is consistent with acute hemorrhagic type pancreatitis, a severe form of pancreatitis with very high mortality rate. This patient has a history of alcoholism, and the empty bottle of alcohol suggest alcohol consumption, a known leading cause of both acute pancreatitis and chronic pancreatitis. Alcohol intoxication is associated with injury of various organs, mostly chronic injuries. Although alcohol may cause sudden death directly, it is usually due to arrhythmia, and post mortem findings are limited except fatty liver. Hepatocellular carcinoma has grossly identifiable liver mass. Sepsis may cause DIC and associated hemorrhagic changes. TTP usually has thrombosis in kidneys. None of these are known to have pancreatic hemorrhage and necrosis.

6. A. Alcohol causes acute pancreatitis and chronic pancreatitis through abnormal activation of zymogen, toxicity to pancreas cells resulting in premature release of zymogens, promoting CCK and secretin release, sphincter of Oddi spasm and subsequent pancreatic duct obstruction, and alter normal blood flows. ADAMTS13 mutation is seen in TTP. Bacterial toxin is associated with sepsis and other forms of infections. Liver failure is seen in diffuse hepatitis and neoplasm, but not pancreatitis. Acute pancreatitis associated with bile duct obstruction by stones are most commonly seen in patient with cholelithiasis, a condition not seen in this patient.

7. C. Acute onset of epigastric pain with elevated lipase or amylase three times above upper limit is diagnostic for acute pancreatitis and the initial treatment is supportive with fluid replacement. Theoretically no additional test is needed. Blood culture is for sepsis. Mesentery angiography is used to diagnose mesentery thrombosis. Upper endoscopy can be used to exam esophageal and stomach abnormalities.

8. D. Cystic lesion in pancreas without epithelial lining is consistent with pseudocyst, a complication of acute pancreatitis. Autoimmune pancreatitis has lymphoplasmacytic infiltrate, acinar atrophy and fibrosis, but not cyst formation. Congenital cyst, mucinous cystic neoplasm, and serous cystadenoma have epithelial lining.

9. C. This patient presents with clinical triad of steatorrhea (fecal fat > 7 g/d), diabetes and pancreatic calcifications, consistent with chronic pancreatitis. Acute pancreatitis has sudden onset. Autoimmune pancreatitis has history of other autoimmune disorders, and usually laboratory tests are positive for at least one of these autoimmune markers, such as IgG4, etc. pancreatic adenocarcinoma or pseudocyst can be detected by image studies.

10. E. Chronic pancreatitis is characterized by pancreatic fibrosis and parenchyma depletion, involving both exocrine and endocrine cells. Cysts with fibrotic wall without epithelial lining is seen in pseudocyst. Diffuse lymphoplasmacytic infiltration with IgG4 positive plasma cells or microabscesses and ductal ulceration are seen in autoimmune pancreatitis. Irregular glands lined by atypical cells are features of adenocarcinoma, including pancreatic adenocarcinoma.

11. B. Diffuse lymphoplasmacytic infiltration of pancreas in a patient with previous history of autoimmune disorder, in this case, chronic sclerosing sialadenitis, is most likely autoimmune pancreatitis. Acute pancreatitis has sudden onset and neutrophilic infiltration, hemorrhagic changes or necrosis. Chronic pancreatitis usually presents with triad of steatorrhea, diabetes and pancreatic calcifications, and is characterized by pancreatic fibrosis and parenchyma depletion, involving both exocrine and endocrine cells. Diffuse large B-cell lymphoma is a B-cell malignancy composed of CD20 positive B cells. Pancreatic adenocarcinoma has irregular glands lined by atypical cells.

12. D. Type 1 autoimmune pancreatitis is the pancreatic manifestation of IgG4 related systemic disease, which may present with chronic sclerosing sialadenitis, as seen in this patient. Elevated CA19-9 may be seen in various mucinous neoplasms, including pancreatic adenocarcinoma, and cholangiocarcinoma, as well as benign conditions including cirrhosis and pancreatitis. CD20 is a B cell marker and can be elevated in various B cell lymphomas, including diffuse large B-cell lymphoma. CEA is elevated in various mucinous neoplasms, including pancreatic adenocarcinoma, ovarian mucinous neoplasms, and colon cancers. Elevated IgM may be seen in association with immune response, or plasma cell neoplasms, such as multiple myeloma, plasmacytoma and monoclonal gammopathy of undetermined significance.

13. D. This patient has presentations of acute pancreatitis. With the early onset and previous history of two episodes of acute pancreatitis and a positive family history of pancreatitis and pancreas cancers and diabetes, this is likely hereditary pancreatitis, commonly associated with gain-of-function mutation PRSS1. Accelerated fat metabolism is associated with diabetic ketoacidosis. Bile duct obstruction is a leading cause of acute pancreatitis, especially in patients with biliary diseases or alcohol usage, but this patient has history of neither. Viral infections usually do not cause recurrent acute pancreatitis.

14. D. Recurrent acute pancreatitis and a positive family history of pancreatitis and pancreas cancers and diabetes are most likely hereditary pancreatitis. Autoimmune pancreatitis has history of other autoimmune disorders, and usually laboratory tests are positive for at least one of these autoimmune markers, such as IgG4, etc. Chronic pancreatitis usually presents with triad of steatorrhea, diabetes and pancreatic calcifications. Diabetic ketoacidosis has more prominent clinical presentations including nausea, vomiting, shallow rapid breathing, and elevated ketone in urine. Pancreatic adenocarcinoma has identifiable mass, not generalized pancreas enlargement and exudate.

15. E. Multilocular pancreatic cystic lesion lined by cuboidal cells without irregular glandular appearance and cytological atypia is most consistent with serous cystadenoma. Acinar cell carcinoma is usually solid with microscopic features of acinar differentiation or solid sheets of relatively monotonous cells. Ovarian serous cystadenocarcinoma commonly has papillary growth with markedly pleomorphic tumors. Mucinous cystic neoplasm are cystic lesion lined by columnar mucin producing cells and has ovarian-like stroma. Pseudocyst is commonly a complication of acute pancreatitis and chronic pancreatitis, characterized by fibrous cystic lesion without epithelial lining.

16. C. Mucinous cystic neoplasm is a cystic lesion lined by columnar mucin producing cells and has ovarian-like stroma. Endometriosis has history of dysmenorrhea. Intraductal papillary mucinous neoplasm has papillary growth, but not ovarian type stroma. Congenital cyst does not have ovarian type stroma. Serous cystadenoma is multilocular pancreatic cystic lesion lined by cuboidal cells without mucin production or ovarian type stroma.

17. A. Mucinous cystic neoplasm is one of the three high risk factors for pancreatic adenocarcinoma, characterized by irregular glands lined by atypical cells with pleomorphism. Chronic pancreatitis usually presents with triad of steatorrhea, diabetes and pancreatic calcifications. Benign mucinous cystic neoplasm is a cystic lesion lined by benign columnar mucin producing cells and has ovarian-like stroma. Intraductal papillary mucinous neoplasm has papillary growth without cytological atypia, unless dysplasia or adenocarcinoma develops. Serous cystadenoma is multilocular pancreatic cystic lesion lined by cuboidal cells without mucin production or atypia.

18. B. Intraductal papillary mucinous neoplasm has papillary growth without cytological atypia, unless dysplasia or adenocarcinoma develops. Pancreatic adenocarcinoma has irregular glands lined by atypical cells. Benign mucinous cystic neoplasm is a cystic lesion lined by benign columnar mucin producing cells and has ovarian-like stroma. Serous cystadenoma is multilocular pancreatic cystic lesion lined by cuboidal cells without mucin production or atypia. Solid-pseudopapillary neoplasm has weakly cohesive, yet relatively monotonous epithelial cells forming pseudopapillary structures without mucin production.

19. A. Solid growth of weakly cohesive relatively monotonous cells with intercellular spaces formation is most compatible with solid-pseudopapillary neoplasm. One feature for it is the cells surrounding vessels are more packed. It is associated with beta catenin mutation. Trypsinogen 1 (PRSS1) mutation is seen in hereditary pancreatitis. SMAD4 mutation can be seen in pancreatic adenocarcinoma, juvenile polyposis and colon cancers. STK11 mutation can be seen in Peutz-Jegher syndrome, breast and colon cancers. VHL mutation is seen in von Hippel-Lindau syndrome.

20. E. Solid growth of weakly cohesive relatively monotonous cells with intercellular spaces formation is most compatible with solid-pseudopapillary neoplasm. One feature for it is the cells surrounding vessels are more packed. It is associated with beta catenin mutation. Pancreatic adenocarcinoma has irregular glands lined by atypical cells. Benign mucinous cystic neoplasm is a cystic lesion lined by benign columnar mucin producing cells and has ovarian-like stroma. Serous cystadenoma is multilocular pancreatic cystic lesion lined by cuboidal cells without mucin production or atypia.

21. C. Pancreatic adenocarcinoma has irregular glands lined by atypical cells and commonly has elevated serum CA19-9. Elevated AFP is seen in yolk sac tumor and any germ cell tumor with yolk sac components, as well as hepatocellular carcinoma. Elevated amylase and lipase can be seen in acute and chronic pancreatitis, as well as acinar cell carcinoma. IgG4 elevation can be seen in autoimmune pancreatitis, chronic sclerosing sialadenitis, and sclerosing retroperitoneal fibrosis.

22. B. Pancreatic adenocarcinoma has irregular glands lined by atypical cells. Acinar cell carcinoma is usually solid with microscopic features of acinar differentiation or solid sheets of relatively monotonous cells. Benign mucinous cystic neoplasm is a cystic lesion lined by benign columnar mucin producing cells and has ovarian-like stroma. Serous cystadenoma is multilocular pancreatic cystic lesion lined by cuboidal cells without mucin production or atypia. Solid-pseudopapillary neoplasm has weakly cohesive, yet relatively monotonous epithelial cells forming pseudopapillary structures without mucin production.

23. A. Tumor composed of sheets of epithelial cells with relatively monotonous nuclei and basophilic cytoplasm and expressing pancreatic enzymes is most consistent with acinar cell carcinoma. Pancreatic adenocarcinoma has irregular glands lined by atypical cells. Islet cell tumor, such as insulinoma, is positive for neuroendocrine markers, including chromogranin and synaptophysin, but negative for enzymes. Serous cystadenoma is multilocular pancreatic cystic lesion lined by cuboidal cells without mucin production or atypia. Solid-pseudopapillary neoplasm has weakly cohesive, yet relatively monotonous epithelial cells forming pseudopapillary structures without mucin production.

24. C. Thickening of gallbladder wall with scattered lymphocytic and plasma cell infiltrates is consistent with chronic cholecystitis. Acute cholecystitis, including gangrenous cholecystitis, has neutrophilic infiltration, and the latter has transmural necrosis. Adenocarcinoma, regardless of location, usually has atypical cells with glandular differentiation.

25. A. Biliary diseases and alcohol are the two leading cause of acute pancreatitis. Other options are not associated with chronic cholecystitis.

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