Scenarios Ch. 19 Pancreas Flashcards
1 A 24-year-old, G2, P1001, woman has a screening ultrasound
that shows polyhydramnios at 17 weeks’ gestation. She
gives birth at term to a boy who on examination has no abnormal
findings. Since birth, he has often vomited after feedings.
An infant radiograph with contrast enhancement shows the
findings in the figure. Laboratory studies show sodium, 130
mmol/L; potassium, 3.4 mmol/L; chloride, 85 mmol/L; CO2,
32 mmol/L; glucose, 65 mg/dL; and amylase, 15 U5/L. What
is the most likely diagnosis?
A Acute pancreatitis
B Annular pancreas
C Chronic pancreatitis
D Islet cell adenoma
E Pancreatic adenocarcinoma
F Pyloric stenosis
1 B When the embryologic ventral and dorsal pancreatic
buds do not rotate and fuse properly, the duodenum can become
encircled by pancreatic tissue, producing an obstruction
called annular pancreas. Depending on the degree of obstruction,
this condition may manifest at birth, in childhood, or in
adulthood. Polyhydramnios should suggest upper gastrointestinal
obstruction in utero. The radiograph shows a region of
duodenal narrowing from the surrounding pancreas. An ultrasound
scan may show the double bubble sign with gastric
and duodenal bulb distention with air proximal to a region of
duodenal obstruction. Acute and chronic pancreatitis should
be accompanied by abdominal pain and by an elevated serum
amylase; they do not generally cause mechanical bowel
obstruction, although the inflammation may cause paralytic
ileus. Neoplasms are focal lesions that may obstruct the biliary
tree and pancreatic duct, but generally do not obstruct the
duodenum. Pyloric stenosis is seen in neonates and early infants,
but generally at 2 to 3 weeks after birth, with projectile
vomiting and a palpable epigastric mass.
2 A 16-year-old boy incurs a gunshot wound to the abdomen
in a drive-by shooting. At exploratory laparotomy, the
surgeon finds a perforated portion of jejunum. She resects
this portion and palpates a mass in the jejunal submucosa.
Sectioning of this lesion reveals that it is a 1.5-cm diameter
circumscribed, solid, tan mass. The mass is sent for frozen
section. What is the pathologist most likely to see under her
microscope?
A Adenocarcinoma
B Adrenal medulla
C Gastric mucosa
D Non-Hodgkin lymphoma
E Pancreatic acini
2 E This incidental finding is consistent with ectopic pancreas,
which can be found in the bowel in 2% of individuals.
It may be found within a Meckel diverticulum, although in
this case no diverticulum was found, and such a diverticulum
would be found in the distal ileum as a vitelline duct remnant.
Adenocarcinoma is unlikely in a boy in his teens, and primary
adenocarcinoma of the small intestine is rare. Adrenal cortical
rests may be found in the pelvis, but usually not bowel. Ectopic
gastric mucosa is most often found within a Meckel diverticulum.
Non-Hodgkin lymphomas of the bowel produce
larger masses and are usually seen in older individuals.
3 A 33-year-old woman with Hodgkin lymphoma of cervical
lymph nodes has an abdominal CT scan for staging. The
scan reveals a solitary 4-cm cyst in the body of the pancreas. A
fine-needle aspirate is performed and yields clear serous fluid
that microscopically has low cuboidal cells with no atypia.
What is this cyst most likely to be?
A Adenocarcinoma
B A component of autosomal dominant polycystic
kidney disease
C Congenital malformation
D Cystadenoma, serous
E Pseudocyst
3 C This is an incidentaloma, or finding with no clinical significance,
other than recognizing that it should be left alone. More
diagnostic testing yields more findings that are not significant
to the health of the patient. Follow the dictum in fishing: catch
and release. Polycystic disease is marked by multiple cysts,
which are also benign, but have a tendency to hemorrhage or
become infected. Without additional cellular proliferative features,
this is not likely to be a neoplasm. A pseudocyst is a complication
of pancreatitis and is lined by granulation tissue.
4 A clinical study of patients with acute abdomen is performed.
Those patients with elevated serum lipase are identified.
A decrease in which of the following analytes is most
likely to predict a worse prognosis?
A Albumin
B Bilirubin
C Calcium
D Fibrinogen
E Haptoglobin
4 C Acute pancreatitis is marked by release of enzymes
that produce chalky areas of fat necrosis, with precipitation
of calcium that lowers serum calcium. The worse the inflammation,
the lower the calcium level. Hypoalbuminemia is
more likely to occur as a complication of chronic pancreatitis
with malabsorption. Hyperbilirubinemia can occur in cases
of gallstone-induced pancreatitis, but the level of bilirubin
does not give an indication of the severity of pancreatitis.
Fibrinogen is an acute phase reactant that increases with
inflammation, but does not correlate well with location or
extent of inflammation. The serum haptoglobin decreases
with hemolysis.
5 A 35-year-old man has a 1-year history of bouts of dull
abdominal pain. Over the next 5 years he also develops steatorrhea
and mild glucose intolerance. He does not develop
jaundice. An abdominal CT scan shows specks of calcification
in the midabdomen, particularly near the duodenum. One of
his sisters has a similar clinical picture, but both parents are
unaffected. Loss of inhibition of which of the following is the
most likely cause for the disease seen in these persons?
A Amylase
B Complement
C Lipase
D Transforming growth factor beta (TGF-β)
E Trypsin
5 E This form of hereditary autosomal recessive pancreatitis
results from abnormal activation of trypsin, causing a
cascade of events leading to acute pancreatitis. The serine protease
inhibitor Kazal type 1 (SPINK1) gene codes for a pancreatic
secretory trypsin inhibitor. Activation of trypsin also leads
to activation of Hageman factor, kallikrein, and complement
that promote the vascular abnormalities in pancreatitis. Both SPINK1 and PRSS1 mutations carry a greatly increased risk
for pancreatic cancer. Both amylase and lipase are released
in pancreatitis as a consequence of trypsin activation. TGF-β
plays a role in the fibrogenesis of chronic pancreatitis.
6 A 52-year-old man has had severe abdominal pain for
the past 2 days. Physical examination shows boardlike rigidity
of the abdominal muscles, making further examination
difficult. There is no observable abdominal distention. The
representative gross appearance of the disease process is
shown in the figure. Which of the following is the mechanism
most likely to produce this appearance?
A CFTR gene mutation
B Coxsackievirus B infection
C Dysregulation of trypsinogen inactivation
D Marked hypertriglyceridemia
E Vasculitis with acute ischemia
F Blunt force trauma to the abdomen
6 C The figure shows acute hemorrhagic pancreatitis with
foci of chalky white fat necrosis. Fundamental to the causation
of acute pancreatitis is inappropriate activation of digestive
enzymes in the acini and the consequent autodigestion of
the pancreas. These enzymes are present as proenzymes in the
acini and are activated by trypsin. Trypsin itself is derived from
trypsinogen, and any abnormality that prevents regulated inactivation
of trypsinogen can lead to excessive trypsin-mediated
activation of other digestive enzymes, such as lipase, amylase,
and elastase. Evidence for this mechanism comes from the
observation that the rare disease hereditary pancreatitis, with
germline mutations that affect a site on the cationic trypsinogen
molecule essential for the cleavage of trypsin by trypsin itself,
results in trypsinogen and trypsin that become resistant to inactivation,
and the abnormally active trypsin activates other
digestive proenzymes, leading to development of pancreatitis.
Pancreatic duct obstruction by gallstones is the most common
event precipitating trypsinogen activation. A CFTR gene mutation
can give rise to chronic pancreatitis, even in the absence of
cystic fibrosis. Viral infections, hypertriglyceridemia, vasculitis,
and trauma are less common causes for pancreatitis.
7 An 11-year-old boy has experienced multiple bouts of severe
abdominal pain for the past 6 years, but no other medical
problems. His father and grandfather have been similarly affected.
On examination during the last episode, bowel sounds
were absent, and he exhibited marked diffuse abdominal pain
with abdominal wall rigidity. A radiograph of the abdomen
showed dilated loops of bowel with air-fluid levels. Laboratory
studies showed serum amylase of 3320 U/L. This boy is
most likely to have an inherited mutation involving which of
the following genes?
A CFTR
B KRAS
C PRSS1
D SMAD4
E SPINK1
7 C This boy has evidence for recurrent pancreatitis, marked
by severe abdominal pain, paralytic ileus, and hyperamylasemia.
An onset of pancreatitis in children suggests a genetic
basis, and the history of an involved parent and grandparent
suggests an autosomal dominant mode of transmission. The
PRSS1 gene encodes for cationic trypsinogen, which, when
mutated, leads to resistance of trypsin to inactivation. The
SPINK1 gene encodes for a trypsin inhibitor, and mutation
can lead to pancreatitis, but the inheritance is autosomal recessive.
A mutation in the CFTR gene can lead to cystic fibrosis,
but there should be pulmonary problems by age 11 years, and
bouts of pancreatitis are usually not severe. KRAS (an oncogene)
and SMAD4 (a tumor suppressor gene) are involved
with development of pancreatic adenocarcinoma.
8 A 38-year-old woman with a long history of gallbladder
disease has a sudden onset of severe midabdominal pain. On
physical examination, she has marked abdominal tenderness,
particularly in the upper abdomen, and bowel sounds are reduced.
An abdominal radiograph shows no free air, but there
is marked soft tissue edema. Abdominal CT scan shows decreased
attenuation with fluid density involving the pancreas.
She is given intravenous fluids and nasogastric suction and
recovers gradually. Which of the following serum laboratory
tests is most useful for diagnosis of her disease process?
A Alanine aminotransferase
B Ammonia
C Bicarbonate
D Bilirubin
E Glucose
F Amylase
8 F The clinical features and the preexisting gallbladder
disease are highly suggestive of acute pancreatitis. This is
confirmed by the appearance of the pancreas on radiologic
imaging. The serum amylase as well as serum and urine lipase
levels are rapidly elevated after an attack of acute pancreatitis.
These enzymes are released from necrotic pancreatic
acini. Abnormalities in liver function test results may be seen
in cases of gallstone pancreatitis but do not confirm pancreatic
involvement. An increased ALT level is more characteristic of
liver cell injury. Hyperammonemia is a feature of liver failure.
Although the pancreatic acinar cells can produce bicarbonate,
levels of this analyte do not correlate well with pancreatitis.
Bilirubin may be elevated with gallstone passage down the
biliary tree, but does not confirm pancreatic disease. In acute pancreatitis, the islets of Langerhans still function, but typically
do not become hyperactive.
9 A 63-year-old man who had worsening congestive heart
failure with cardiac dysrhythmias for the past year of his life
died of pneumonia. At autopsy, his pancreas is grossly small
and densely fibrotic. Microscopic examination shows extensive
atrophy of the acini with residual chronic inflammation, fibrosis,
and inspissated protein plugs in small, obstructed pancreatic
ducts. Some of the protein plugs show calcification. The
islets of Langerhans appear normal. The heart weighs 500 g,
and all four chambers are dilated. Which of the following conditions
is most likely to account for his findings?
A αl-Antitrypsin deficiency
B Blunt trauma to the abdomen
C Cholelithiasis
D Chronic alcoholism
E Cystic fibrosis
F Hypercholesterolemia
9 D This patient has chronic pancreatitis. Alcohol promotes
intracellular proenzyme activation that leads to acinar cell
injury. Although the exact mechanism for development of
pancreatitis with alcoholism is unknown, the production of a
protein-rich pancreatic secretion that forms inspissated plugs
that cause ductal obstruction is observed in many cases. Ductal
obstruction predisposes to acinar injury, and the ongoing or
repeated injury leads to chronic pancreatitis. Patients with a
history of alcohol abuse may have bouts of chronic pancreatitis
that go unnoticed, only to have a superimposed case of clinically
apparent acute pancreatitis. Alcohol, drugs, traumatic
injury, and viral agents may have a direct injurious effect on
pancreatic exocrine acinar cells. A dilated cardiomyopathy also
can occur in chronic alcoholism, as in this case. A deficiency of
α1-antitrypsin can produce liver disease with chronic hepatitis
or cirrhosis, or both. Blunt abdominal trauma can produce
hemorrhage, sometimes with a component of acute pancreatitis.
Cystic fibrosis decreases bicarbonate excretion and promotes
protein plugging of ducts, but the appearance is more
of a subclinical chronic pancreatitis, with fat and fat-soluble
vitamin malabsorption, and depending upon the CFTR mutation,
other features of cystic fibrosis may be lacking in these
patients. Hypercholesterolemia (typically at least 1000 mg/dL
or more) can cause acute pancreatitis, as can cholelithiasis.
10 A 39-year-old man has had numerous bouts of pneumonia
caused by Pseudomonas aeruginosa and Burkholderia cepacia for
the past 35 years. He now has diarrhea of mild-to-moderate volume.
On physical examination, he has decreased breath sounds
and dullness to percussion in both lungs. His stool guaiac test is
negative. Laboratory studies show the ΔF508 mutation. His quantitative
stool fat is 7.5 g/day. Which of the following pathologic
findings is most likely to be present in the pancreas of this patient?
A Acute inflammation
B Acinar atrophy
C Adenocarcinoma
D Amyloidosis
E Pseudocyst
10 B Cystic fibrosis is an autosomal recessive condition that
results from an abnormal cystic fibrosis transmembrane conductance
regulator (CFTR) gene. The most common mutation
is ΔF508. There are abnormal viscid secretions affecting the
pancreas. This results in ductal obstruction, and this leads to
a form of chronic pancreatitis with acinar atrophy. Eventually,
the exocrine function is gone. Clinically symptomatic, florid
acute and chronic pancreatitis and the complication of inflammation
known as a pseudocyst do not typically occur in this
setting. Cystic fibrosis is not a risk factor for adenoma or carcinoma
of the pancreas. Amyloid deposition may be seen in the
islets of Langerhans in a patient with type 2 diabetes mellitus,
but generalized amyloid deposition of the pancreas is rare.
11 A 36-year-old woman with a history of pulmonary infections
since childhood has had chronic abdominal pain for 4
years, along with a 6-kg weight loss. On physical examination
her BMI is 18. There is pitting edema on the lower leg. Laboratory
studies show serum albumin 3 g/dL, total protein 5.2 g/
dL, and glucose 155 mg/dL. Abdominal CT imaging shows
calcifications in the upper abdomen, posterior to the stomach.
No mass lesion is noted. She is most likely to have a mutation
involving which of the following genes?
A CFTR
B KRAS
C PRSS1
D SPINK1
E VHL
11 A She has chronic pancreatitis. One could reflexively
blame this on alcohol abuse, but the real cause can be obscure,
and many cases of pancreatitis have been termed idiopathic.
Genetic testing may reveal a mutation in the CFTR
gene. There are hundreds of such mutations, and not all of
them are associated with pulmonary disease, but may have
other clinical manifestations, such as pancreatitis. KRAS mutations
are found with adenocarcinomas. PRSS1 and SPINK1
mutations are associated with hereditary pancreatitis as well
as increased risk for pancreatic carcinoma, but neither is associated
with lung disease. VHL gene mutations may be associated
with serous cystadenoma.
12 A 65-year-old woman has had upper abdominal pain for
the past month. On examination, the pain is localized to the
epigastric region on palpation. Abdominal CT scan shows a
well-circumscribed, 8-cm mass in the tail of the pancreas that
has many small fluid-filled areas. At laparotomy, the mass is
removed and on microscopic examination shows glycogenrich,
low cuboidal cells surrounding spaces filled with clear
fluid. There is no recurrence of the lesion. What is the most
likely diagnosis?
A Adenocarcinoma
B Autosomal dominant polycystic kidney disease
C Chronic pancreatitis
D Cystic fibrosis
E Pseudocyst
F Serous cystadenoma
12 F Most pancreatic cysts are nonneoplastic, such as
pseudocysts and the scattered cysts associated with autosomal
domi nant polycystic kidney disease (ADPKD). It is unlikely
that ADPKD would involve pancreas in the absence
of very large multicystic kidneys. This woman has a solitary
mass with features of a benign serous cystadenoma. Adenocarcinomas
are more aggressive neoplasms that are rarely
cured by surgery because they are large and infiltrative at
the time of diagnosis; they tend to be solid masses, although
there can be areas of necrosis. Chronic pancreatitis leads to
fibrosis. Cystic fibrosis can lead to chronic pancreatitis. The
pancreas is usually small and fibrotic with chronic pancreatitis.
A pseudocyst usually forms in the setting of extensive
pancreatic destruction with acute inflammation, typically
acute pancreatitis, or acute inflammation superimposed on
chronic inflammation with chronic alcoholism.
13 A 46-year-old woman has severe abdominal pain for
2 days. On physical examination she has marked epigastric
pain, and bowel sounds are reduced. Laboratory studies show
an elevated serum lipase. With supportive care, her acute condition
subsides within 7 days. Which of the following complications
is most likely to occur in this patient?
A Gastric ulceration
B Hemoperitoneum
C Hyperosmolar coma
D Ketoacidosis
E Pseudocyst formation
F Small bowel infarction
13 E During acute pancreatitis, the extent of necrosis may
be so severe that a liquefied area becomes surrounded by
granulation tissue, forming a cystic mass. Because there is no
epithelial lining in the cyst, however, it is best called a pseudocyst.
Although the pancreas is inferior and posterior to the
stomach, spread of inflammation to the stomach does not typically
occur. Although acute pancreatitis may be hemorrhagic,
the hemorrhage is confined to the body of the pancreas and
surrounding fibroadipose tissue. The islets of Langerhans
usually continue to function despite marked inflammation
of the parenchyma. Lack of insulin is not a typical feature of
pancreatitis. The inflammation is unlikely to compromise the
blood supply to abdominal organs and produce an infarction.
14 A 58-year-old woman has experienced abdominal discomfort
for the past year. On physical examination, she has midepigastric
tenderness on palpation. Laboratory studies show a normal
serum lipase. The figure shows her abdominal CT scan. The lesion
is excised, and on microscopic examination has cells that show cytologic
and architectural atypia, but no invasion of surrounding
pancreatic parenchyma. What is the most likely diagnosis?
A Adenocarcinoma
B Autosomal dominant polycystic kidney disease
C Mucinous cystic neoplasm
D Cystic fibrosis
E Islet cell tumor, nonfunctional
F Pseudocyst
14 C The CT scan shows a circumscribed, multilocular,
cystic mass. Mucinous tumors of the pancreas can be completely
benign, borderline, or malignant. The presence of
cytologic and architectural atypia (low-grade dysplasia) indicates
the lesion is not completely benign, but in the absence of
clearly malignant features of invasion or metastasis, the lesion
fits in the borderline category. Such “in between” terminology
frustrates both health care providers and their patients,
but there are often no simple answers when dealing with
neoplasms. However, over time an adenocarcinoma can arise
within a mucinous cystic neoplasm. Pancreatic adenocarcinomas
are more aggressive solid neoplasms that have an overall
poor prognosis. The cysts of ADPKD are benign and scattered
throughout the pancreas without forming a mass effect; the
kidneys in this CT scan are not cystic. Cystic fibrosis can lead
to chronic pancreatitis, not neoplasia of the pancreas. Islet cell
tumors are solid masses that are usually quite small when benign
and sometimes associated with hormone production. A
pseudocyst is an inflammatory structure arising in the setting
of pancreatitis with extensive necrosis; it is nonneoplastic.
15 A 79-year-old woman belongs to a religious sect that follows
the dictum “if it sounds like fun, you shouldn’t do it,” and
has in so (not) doing avoided many risk factors for disease and
remained healthy. For the past 7 months, she has had vague
abdominal pain, however, and yesterday she experienced
acute chest pain with dyspnea. Chest and abdomen CT scans
show a pulmonary embolus. Her abdominal CT scan is shown
in the figure. Laboratory studies show elevation in CEA and
CA19-9. A mutation involving which of the following genes is
most likely implicated in development of this mass?
A APC
B BRCA2
C CFTR
D KRAS
E PRSS1
F SPINK1
15 D Mortality happens regardless. Prevalence for sporadic
cancer (no identifiable inherited risks) increases with age.
The figure shows an irregular mass occupying most of the
body and tail of the pancreas, along with hepatic metastases. She has a pancreatic adenocarcinoma with Trousseau syndrome
(a paraneoplastic hypercoagulable state) leading to
pulmonary thromboembolism. The tumor markers CEA and
CA19-9 are often present, but insensitive for early diagnosis
of pancreatic cancer. KRAS mutations are found in more than
90% of pancreatic adenocarcinomas. APC gene mutations
are associated with hereditary and sporadic forms of colonic
adenocarcinoma. BRCA2 mutations are found in some
pancreatic cancers, but usually there is a history of familial
breast and ovarian cancers. CFTR mutations are associated
with cystic fibrosis, which is not a risk for pancreatic cancer.
PRSS1 mutations carry a risk for pancreatic adenocarcinoma,
but there should be a history of pancreatitis starting early in
life. SPINK1 mutations are also associated with hereditary
pancreatitis, and a significant risk for pancreatic cancer.
