SM_160b: Clinical Features of Pancreatic Disorders

Question 1

___ is inflammation of the pancreas that occurs when enzymes of the pancreas auto-digest the pancreas

Answer 1

Pancreatitis is inflammation of the pancreas that occurs when enzymes of the pancreas auto-digest the pancreas

• Acute pancreatitis: occurs suddenly, short-term, can resovle
• Chronic pancreatitis: irreversible changes of the pancreas

Question 2

Describe diagnosis of acute pancreatitis

Answer 2

Acute pancreatitis: need 2 of 3, avoid imaging unless diagnosis is in question

• Symptoms: classically with severe epigastric pain radiation to back
• Labs: amylase or lipase > 3x upper limit of normal
• Radiology suggestive of pancreatitis

Question 3

____ and ____ are enzymes mainly but not exclusively produced by the pancreas and released into the small intestine via the pancreas duct

Answer 3

Lipase and amylase are enzymes mainly but not exclusively produced by the pancreas and released into the small intestine via the pancreas duct

• There are normal circulating levels

Question 4

____ is an enzyme that helps break down fats in the diet

Answer 4

Lipase is an enzyme that helps break down fats in the diet

Question 5

____ is an enzyme that helps break down carbohydrates

Answer 5

Amylase is an enzyme that helps break down carbohydrates

Question 6

Describe definitions of acute pancreatitis

Answer 6

Acute pancreatitis

• Interstitial edematous pancreatitis: inflammation of the pancreatic parenchyma and peripancreatic tissues w/o necrosis
• Necrotizing pancreatitis: inflammation associated with parenchymal or periparenchyma necrosis (death of cells)
• Mild: absence of organ failure or local / systemic complications
• Moderately severe: transient organ failure and/or local system complications (< 48 hours)
• Severe: persistent organ failure of ≥ 1 organs

Question 7

Diagnosis of pancreatitis should be AVOIDED with ____

Answer 7

Diagnosis of pancreatitis should be AVOIDED with nonspecific elevations of amylase or lipase

Question 8

Describe diagnosis of chronic pancreatitis

Answer 8

Chronic pancreatitis diagnosis

• Radiology and endoscopic tests
• Clinical symptoms (specifically pain and malabsorption)
• Diagnostic testing (to measure pancreas function)

Question 9

Chronic pancreatitis has ____ in presentation

Answer 9

Chronic pancreatitis has marked heterogeneity in presentation

• Incidental symptomatic severe chronic pancreatitis
• Symptomatic severe chronic pancreatitis
• After ≥ 1 prior episodes of acute pancreatitis
• Subtle signs (fat malabsorption)

Question 10

Acute pancreatitis etiology is commonly ____ or ____

Answer 10

Acute pancreatitis etiology is commonly alcohol or biliary

• Alcohol: dose-dependent risk for pancreatitis
• Biliary: gallstones, sludge, microlithiasis

Question 11

Acute pancreatitis genes include ____, ____, and ____

Answer 11

Acute pancreatitis genes include PRSS1, CFTR, and SPINK1

• PRSS1 gene encodes cationic trypsinogen: mutations result in autosomal dominant inheritance of hereditary pancreatitis
• CFTR mutations: transmitted in autosomal recessive fashion
• SPINK1 mutations: generally disease modifying, autosomal recessive

Question 12

Autoimmune pancreatitis is most likely ____ mediated, diagnosed by ____, and presents as ____

Answer 12

Autoimmune pancreatitis is most likely IgG4 mediated, diagnosed by elevated serum IgG4, and presents as painless mass

• Treated with prolonged steroids

Question 13

Pancreatitis may occur after ____ due to papillary swelling or contrast injection

Answer 13

Pancreatitis may occur after endoscopic retrograde cholangiopancreatography (ECRP) due to papillary swelling or contrast injection

• Magnetic resonance cholangiopancreatography has replaced ECRP
• Placement of a small pancreatic stent in at-risk patients reduces the risk of post-ERCP pancreatitis
• Indomethacin reduces risk of post-ERCP pancreatitis
• Aggressive IC hydration after ERCP may reduce post-ERCP pancreatitis

Question 14

Describe diagnosis of acute pancreatitis

Answer 14

Acute pancreatitis diagnosis

• All patients should be ruled out for biliary causes regardless of alcohol history: ALT elevation is most sensitive
• RUQ ultrasound is necessary
• CT should be avoided when diagnosis is known
• MRCP cam be useful but not needed at first episode to evaluate for other causes

Question 15

Describe diagnosis of recurrent acute pancreatitis

Answer 15

Recurrent acute pancreatitis diagnosis

• Patients with normal liver tests on hospital day #1 and no sludge in gallbladder points away from a biliary source
• Pancreas divisum should be considered
• MRI/MRCP should be considered to evaluate for pancreas divisum, US can be used to evaluate for divisum
• Consider med history, autoimmune pancreatitis, genetic testing

Question 16

Describe acute pancreatitis management

Answer 16

Acute pancreatitis management

• IV hydration
• Nutrition: avoidance of oral nutrition, but parenteral nutrition has drawbacks, nasojejundal feedings superior to parenteral nutrition
• No advantage to early feeding

Question 17

Early complications of acute pancreatitis include ____ or ____

Answer 17

Early complications of acute pancreatitis include acute pancreatic fluid collections or acute necrosis collections

• Acute pancreatic fluid collections: due to inflammation and/or pancreas duct disruption leading to accumulation of pancreatic juice, most resolve on their own
• Acute necrosis collections: aspiraation can be performed, avoid surgery early in necrosis

Question 18

Acute pancreatitis complications after 4 weeks include ____ or ____

Answer 18

Acute pancreatitis complications after 4 weeks include pseudocyst or walled off pancreatic necrosis

• Pseudocysts: mature collections of pancreas juice possibly associated with persistent disruption of pancreatic duct, drain larger pseudocysts
• Walled off pancreatic necrosis: treat if infected or symptomatic, debridement and drainage

Question 19

____ is a risk factor for chronic pancreatitis

Answer 19

Tobacco is a risk factor for chronic pancreatitis

Question 20

___ significantly increases the risk of developing pancreatic cancer

Answer 20

Smoking significantly increases the risk of developing pancreatic cancer

Question 21

Chronic pancreatitis presents as asymptomatic or with ____, ____, ____, as ____

Answer 21

Chronic pancreatitis presents as asymptomatic or with exocrine insufficiency (malabsorption), endocrine insufficiency (diabetes mellitus), pancreatic pain, and neuropathic pain

Question 22

Describe treatment of chronic pancreatitis

Answer 22

Chronic pancreatitis treatment

• Pancreas enzyme replacement therapy used to treat exocrine function: dosage measured in units of lipase
• Opiates should be avoided

Question 23

Solid exocrine pancreas neoplasm is ____

Answer 23

Solid exocrine pancreas neoplasm is ductal adenocarcinoma

Question 24

Solid endocrine pancreas neoplasm is ____

Answer 24

Solid endocrine pancreas neoplasm is iselt cell neoplasms

Question 25

Benign cystic pancreas neoplasms are ____, ____, and ____

Answer 25

Benign cystic pancreas neoplasms are retention cyst, pseudocyst, and serous cystadenoma

Question 26

Malignant potential cystic pancreas neoplasms are ____, ____, and ____

Answer 26

Malignant potential cystic pancreas neoplasms are mucinous cystadenoma, intraductal papillary mucinous neoplasm, and solid pseudopapillary neoplasm

Question 27

____ is the third leading cause of cancer death

Answer 27

Pancreatic adenocarcinoma is the third leading cause of cancer death

• Risk factors: male gender, family history, cigarette smoking, cancer syndromes, non-O blood type, chronic pancreatitis, and obesity / decreased physicla activity
• Genetic syndromes: Peutz-Jeghers (STK11), familial atypical multiple melanoma (FAMM, CDKN2A), BRCA1, BRCA2, and Lynch syndrome

Question 28

Describe pancreatic cancer presentation

Answer 28

Pancreatic cancder presentation

• Tumors in pancreas head block the bile duct earlier and cause jaundice
• Tumors in the body and tail generally present with advanced disease

Question 29

____ is the only chance of cure for pancreatic adenocarcinoma but overall survival is low

Answer 29

Surgical resection is the only chance of cure for pancreatic adenocarcinoma but overall survival is low

Question 31

Pancreatic endocrine tumors are named after ____

Answer 31

Pancreatic endocrine tumors are named after the hormone they secrete

• Insulinomas
• Gastrinomas
• Nonfunctioning
• Glucagonomas
• Somatistatinomas
• VIPomas

Question 32

Pancreatic neuroendocrine tumors may be classified as ____ or ____

Answer 32

Pancreatic neuroendocrine tumors may be classified as functional or nonfunctional

• Functional: found at earlier stage due to symptoms
• Nonfunctioning: often incidentally found on CT performed for other reasons

Question 33

Insulinomas are characterized by ____ and diagnosed by ____

Answer 33

Insulinomas are characterized by fasting hypoglycemia and diagnosed by high insulin and c-peptide levels after a prolonged fast (i.e. 72 hour fast)

Question 34

Gastrinomas most commonly arise in the ____ and have signs due to ____

Answer 34

Gastrinomas most commonly arise in the duodenum and have signs due to elevated gastrin levels

• Remainder found in pancreas or peri-pancreatic lymph nodes
• Signs: abdominal pain, diarrhea, enlarged gastric folds, peptic ulcer disease
• Diagnosis: fastin serum gastrin level (requires acidic gastric pH), secretin stimulation test (gastrin levels rise > 200 after secretin administration)

Question 35

Mucinous cystic neoplasms occur almost exclusively in ____ around age ____

Answer 35

Mucinous cystic neoplasms occur almost exclusively in women around age 40 (mother)

• Generally behave more aggressively than other pancreas cysts and resection is recommended

Question 36

____ indicates the presence of a mucinous cyst neoplasm

Answer 36

Ovarian-like stroma indicates the presence of a mucinous cyst neoplasm

• Generally macrocystic and located in body and tail
• Lesiosn do not communicate with the pancreatic duct
• Generally asymptomatic

Question 37

Intraductal papillary mucinous neooplasms are more common in ____ and typically occur in ____

Answer 37

Intraductal papillary mucinous neooplasms are more common in men and typically occur in elderly (grandparent)

• Malignant potential is less than mucinous cystic neoplasms

Question 38

____ is pathognomonic for intraductal papillary mucinous neoplasms

Answer 38

Mucin extruding through major papilla is pathognomonic for intraductal papillary mucinous neoplasms

• Mucin-producing cells arranged in a papillary pattern
• Communicates with pancreatid cut: main, side-branch, or both

Question 39

Serous cystadenomas are more common in ____, present in ____, and are often found ____

Answer 39

Serous cystadenomas are more common in women, present in middle age to elderly, and are often found asymptomatically

• Very low malignant potential
• Resection recommended for symptomatic cysts, cysts which cannot be differentiated from mucinous cystic neoplasms, rapidly growing cysts