SM_159b: Pathology of Pancreatic Disorders

Question 1

Pancreas regions are the ____, ____, ____, and ____

Answer 1

Pancreas regions are the head, neck, body, and tail

Question 2

Uncinate process of the pancreas hooks around the ____ and ____

Answer 2

Uncinate process of the head of the pancreas hooks around the superior mesenteric artery and superior mesenteric vein

Question 3

Superior mesenteric artery and vein run behind the ____ of the pancreas

Answer 3

Superior mesenteric artery and vein run behind the body of the pancreas

Question 4

Exocrine component of the pancreas is ____ and ____

Answer 4

Exocrine component of the pancreas is acini and ducts

(acini make up most of pancreas)

Question 5

Endocrine component of the pancreas is the ____

Answer 5

Endocrine component of the pancreas is the islets of Langerhans

Question 6

Acini of pancreas ____

Answer 6

Acini of pancreas produce, store, and secrete digestive enzymes (amylase, lipase, trypsin, etc)

Question 7

Ducts of pancreas ____ and ____

Answer 7

Ducts of pancreas carry the enzymes to duodenum and secrete mucin for lubrication, activation, and protection

Question 8

Describe the exocrine pancreas

Answer 8

Exocrine pancreas

• Majority of pancreatic enzymes are synthesized by inactive proenzymes and sequestered in membrane-bound zymogen granules
• Activation of pro-enzymes requires conversion of trypsinogen to trypsin by duodenal enteropeptidase
• Trypsin inhibitors are also secreted by acinar and ductal cells
• Trypsin cleaves and inactivates itself: negative feedback mechanism that normally limits local levels of activated trypsin

Question 9

Endocrine pancreas produces the hormones ____, ____, ____, and ____

Answer 9

Endocrine pancreas produces the hormones insulin, glucagon, somatostatin, and pancreatic polypeptide

Question 10

Acute pancreatitis is ____

Answer 10

Acute pancreatitis is reversible inflammatory disorder that varies in severity from focal edema and fat necrosis to widespread hemorrhagic necrosis

Question 11

Pancreatitis pathogenesis is ____

Answer 11

Pancreatitis pathogenesis is autodigestion of the pancreas by inappropriately activated pancreatic enzymes

• Premature activation of trypsin within the substance of pancreas can unleash other proenzymes -> acute pancreatitis

Question 12

____ and ____ commonly cause acute pancreatitis

Answer 12

Cholelithiasis and alcohol commonly cause acute pancreatitis

Question 13

Describe clinical features of acute pancreatitis

Answer 13

Acute pancreatitis clinical features

• Adults primarily
• Alcoholics (more commonly men) and gallstones (more commonly women)
• Laboratory tests: elevated amylase and lipase

Question 14

Acute pancreatitis commonly presents with ____, ____, and ____

Answer 14

Acute pancreatitis commonly presents with acute upper abdominal pain, nausea, and vomiting

Question 15

Describe pathology of acute pancreatitis

Answer 15

Acute pancreatitis pathology

• Gross findings depend on severity of disease
• Usually, the gland is swollen and edematous
• Fat necrosis appears as whitish yellow plaques
• Hemorrhagic pancreatitis: pancreas is dark brown

Question 16

This is ____ in ____

Answer 16

This is fat necrosis in acute pancreatitis

Question 18

Severe necrotizing pancreatitis is associated with significant mortality and high complication rates including ____ and ____

Answer 18

Severe necrotizing pancreatitis is associated with significant mortality and high complication rates including pancreatic abscess and pseudocyst

• Mild cases usually recover within 5-7 days
• Treatment: supportive care, infection prevention

Question 19

Pseudocyst is ____

Answer 19

Pseudocyst is a cyst with a collection of fluid and cell debris

• Most common type of cystic lesion in the pancreas

Question 20

Pancreatic pseudocyst cyst wall consists of ____

Answer 20

Pancreatic pseudocyst cyst wall consists of fibrous tissue but no epithelial lining

Question 21

Chronic pancreatitis is characterized by ___

Answer 21

Chronic pancreatitis is characterized by long-standing inflammation that leads to irreversible destruction of the exocrine pancreas followed by loss of iselts of Langerhans

Question 22

Chronic pancreatitis most commonly results from ____

Answer 22

Chronic pancreatitis most commonly results from long-term alcohol abuse

Question 23

Chronic pancreatitis involves ____, ____, ____, ____, ____, and ____

Answer 23

Chronic pancreatitis involves abdominal pain, steatorrhea, diabetes mellitus, weight loss, nausea, and vomiting

Question 24

Describe pathology of chronic pancreatitis on gross examination

Answer 24

Chronic pancreatitis on gross examination

• Pancreas is shrunken and hard
• Cut surface is fibrotic and whitish
• Ducts dilated and may contain calculi

Question 25

Chronic pancreatitis on microscopic examination involves \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 25

Chronic pancreatitis on microscopic examination involves fibrosis, chronic inflammation, and loss of exocrine (acini) and endocrine tissue

Question 26

Describe complications of chronic pancreatitis

Answer 26

Chronic pancreatitis complications * Maldigestion of carbohydrates, fats, and proteins because of atrophy of acinar tissue * Diabetes because of loss of endocrine tissue * Pseudocyst * Obstruction of common bile duct * Slight increase in pancreatic cancer

Question 27

\_\_\_\_ is the fourth leading cause of cancer-related death

Answer 27

Pancreatic ductal adenocarcinoma is the fourth leading cause of cancer-related death

Question 28

Pancreatic ductal carcinoma most commonly occurs between age ____ and most cases are ____ at presentation

Answer 28

Pancreatic ductal carcinoma most commonly occurs between age 60-80 and most cases are unresectable at presentation

Question 29

Pancreatic ductal adenocarcinoma often presents with nonspecific symptoms including \_\_\_\_, \_\_\_\_, and ____ that delay diagnosis

Answer 29

Pancreatic ductal adenocarcinoma often presents with nonspecific symptoms including painless jaundice, abdominal pain, and weight loss that delay diagnosis

Question 30

Pancreatic ductal adenocarcinoma is associated with \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 30

Pancreatic ductal adenocarcinoma is associated with * Trosseau syndrome / migratory thrombophlebitis * Diabetes mellitus * Sister Mary Joseph sign (palpable periumbilical nodules) * Courvoisier sign (distended palpable gallbladder)

Question 31

Pancreatic ductal adenocarcinoma is a \_\_\_\_

Answer 31

Pancreatic ductal adenocarcinoma is a solid infiltrative mass * Larger neoplasms can have necrosis and secondary cystic changes

Question 32

Pancreatic ductal adenocarcinoma histology is characterized by \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 32

Pancreatic ductal adenocarcinoma histology is characterized by lack of lobularity, haphazardness of ductal elements, perineural invasion, and ducts in vascular spaces (carcinoma)

Question 33

Commonly mutated genes in pancreatic ductal adenocarcinoma are \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 33

Commonly mutated genes in pancreatic ductal adenocarcinoma are * KRAS (oncogene): growth factor signal transducer * p16/CDKN2A (tumor suppressor gene): negative cell cycle regulator * TP53 (tumor suppressor gene): response to DNA damage * SMAD4: TGF-beta pathway

Question 35

Describe the pancreatic intraepithelial neoplasm (PanIN) tumor progression model

Answer 35

Pancreatic intraepithelial neoplasm (PanIN) tumor progression model 1. K-ras mutation and telmore shortening to PanIN 1 (low grade dysplasia) 2. p16 inactivation to PanIN 2 3. p53, DPC4, and BRCA2 mutations to PanIN 3 (high grade dysplasia) 4. Invasive carcinoma

Question 36

Intraductal papillary mucinous neoplasm is \_\_\_\_, occurs more frequently in \_\_\_\_, and involves the ____ of the pancreas

Answer 36

Intraductal papillary mucinous neoplasm is a precursor lesion of pancreatic ductal adenocarcinoma, occurs more frequently in men, and involves the head of the pancreas

Question 37

Mucinous cystic neoplasm is \_\_\_\_, occurs in \_\_\_\_, occurs in the ____ of the pancreas, and is a \_\_\_\_

Answer 37

Mucinous cystic neoplasm is a precursor lesion of pancreatic ductal adenocarcinoma, occurs in peri-menopausal women, occurs in the tail of the pancreas, and is a thick walled multilobular cyst * Cysts lined by tall columnar mucin-producing epithelium * Ovarian-type stroma

Question 38

Well-differentiated neuroendocrine cocurs in patients \_\_\_\_, has etiology of ____ or \_\_\_\_, and can be ____ or \_\_\_\_

Answer 38

Well-differentiated neuroendocrine cocurs in patients 30-60 years old, has etiology of sporadic or syndromic diseases, and is functional or nonfunctional * Syndromic diseases: MEN1, von Hippel-Lindau syndrome, tuberous sclerosis * Functional: insulinoma, glucagonoma, somatostatinoma

Question 39

Well-differentiated neuroendocrine tumor conventional architectural features include \_\_\_\_, \_\_\_\_, \_\_\_\_, and \_\_\_\_

Answer 39

Well-differentiated neuroendocrine tumor conventional architectural features include trabeculae, fibrosis, nests, and pseudorosettes

Question 40

Well-differentiated neuroendocrine tumor conventional cytomorphologic is \_\_\_\_

Answer 40

Well-differentiated neuroendocrine tumor conventional cytomorphologic is monotonous cells with round nuclei displaying salt and pepper chromatin and inconspicuous nucleoli

Question 41

Well-differentiated neuroendocrine tumor prognosis is \_\_\_\_

Answer 41

Well-differentiated neuroendocrine tumor prognosis is variable * Features associated with adverse outcome include: high mitosis / high proliferation index, vascular invasion, tumor necrosis

Question 42

Describe common disease of the pancreas

Answer 42

Common disease of the pancreas * Non-neoplastic: acute pancreatitis, chronic pancreatitis * Neoplastic: exocrine (pancreatic ductal adenocarcinoma), endocrine (well-differentiated neuroendocrine tumor)