Exocrine pancreas disorders Flashcards
Pancreas cell that secrete proenzymes
Acinar cells
Parts of pancreas that develop from the ventral bud
Uncinate process and inferior part of head
Genetic anomalies associated with annular pancreas
Trisomy 21
Tracheoesophageal fistula
Cardiac anomalies
Most common congenital anomaly of pancreas
Pancreas divisum
Failure of fusion of the dorsal and ventral pancreatic buds leading to the majority of the pancreas draining into the duodenum through minor papilla
Pancreas divisum
Caused by failure of part of the ventral bud of the pancreas to undergo atrophy, or by aberrant migration
Ectopic pancreas
Most common sites of ectopic pancreas
Stomach
Duodenum
Initiated by injuries that lead to auto-digestion of the pancreas, when protective mechanisms are disrupted or overwhelmed
Pancreatitis
Metabolic causes of acute pancreatitis
Alcoholism
Hypertriglyceridemia
Hypercalcemia
Drugs
Mechanical causes of acute pancreatitis
Gallstones
Trauma/surgery
Iatrogenic injury
ERCP
Vascular causes of acute pancreatitis
Shock
Atheroembolism
Vasculitis (polyarteritis nodasa)
Genetic mutations that can cause acute pancreatitis
PRSS1 –> trypsin activation
SPINK1 –> trypsin inhibitor
CASR –> Ca receptor
CFTR –> cystic fibrosis
Infectious causes of acute pancreatitis
Mumps
Coxsackie virus
Gross appearance of fat necrosis in pancreatitis
Yellow-white chalky areas
FAs combine with Ca to form insoluble soaps creating a granular blue microscopic appearance in necrotic fat cells.
Saponification of fat necrosis in pancreatitis
Pt presents with recent onset constant and intense abdominal pain referred to upper back and L shoulder with anorexia, nausea, and vomiting.
Acute pancreatitis
Serous, slightly turbid, brown-tinged fluid with fat globules in the peritoneal cavity, reflect digestion of adipose tissue
Systemic lipase release causing fat necrosis, associated with pancreatitis
Signs associated with hemorrhagic pancreatitis
Gray-Turner sign –> flank hemorrhage
Cullen sign –> periumbilical hemorrhage
Cause of tetany in acute pancreatitis
Hypocalcemia because of Ca used in saponification of fat necrosis
Additional conditions that can be caused by acute pancreatitis
DIC
Shock
ARDS
Tetany
Serum amylase findings in acute pancreatitis
Markedly increased for first 24 hrs
Normal in 3-5 days
Serum lipase findings in acute pancreatitis
Elevated during 72-96 hrs
Complications of acute pancreatitis
Pancreatic pseudocyst
Pancreatic abscess
Acute kidney failure
Cause of pancreatic pseudocyst in acute pancreatitis
Persistent elevation of serum amylase
Cause of pancreatic abscess in acute pancreatitis
Infection with gram negative organisms
Cause of acute kidney failure in acute pancreatitis
Acute tubular necrosis
Irreversible destruction of exocrine parenchyma and fibrosis, and loss of endocrine parenchyma in the pancreas
Chronic pancreatitis
Most common cause of chronic pancreatitis
Long term alcohol use
Causes of chronic pancreatitis
Alcoholism
Chronic pancreatic duct obstruction (calculi or neoplasm)
Autoimmune injury
Hereditary factors
Chronic pancreatitis associated with a manifestation of systemic IgG related disease
Type 1 autoimmune pancreatitis
Histology shows swirling or storiform fibrosis and obliterative inflammation of the veins within the pancreas
Type 1 autoimmune pancreatitis
Dense lymphoplasmacytic inflammation of the pancreas that is enriched in IgG4 secreting plasma cells
Lymphoplasmacytic sclerosing pancreatitis associated with type 1 autoimmune pancreatitis
Chronic pancreatitis characterized by neutrophilic infiltrates within the epithelium and lumen of medium-sized pancreatic ducts
Type 2 autoimmune pancreatitis
Gross morphology of chronic pancreatitis
Gland is hard, sometimes with visibly dilated duct containing calcified concretions.
Cause of chronic pancreatitis associated with ductal dilation and intraluminal protein plugs and calcifications
Alcohol abuse
Microscopy shows chronic inflammatory infiltrate that surrounds lobules and ducts, with sparing of the islets of Langerhans
Chronic pancreatitis
Microscopy shows extensive fibrosis and atrophy, dilated duct with inspissated concretions, and lymphoplasmacytic infiltrates.
Chronic pancreatitis
CT scan findings of chronic pancreatitis
Dystrophic calcifications within the pancreas
Complications of chronic pancreatitis
Pancreatic exocrine insufficiency
Chronic malabsorption
DM type 1
Pancreatic pseudocyst
Pancreatic carcinoma
Congenital pancreatic enzyme deficiencies
Co-lipase deficiency
Lipase deficiency
Trypsinogen deficiency
Two main functions of exocrine pancreas
Secretion of digestive enzymes
Secretion of bicarbonate-rich fluid, to neutralize gastric acid
What is a sign that pancreatic lipase secretion is reduced by >90%?
Steatorrhea
What is azotorrhea?
Protein malabsorption
Area of intra- or peri-pancreatic hemorrhagic fat necrosis walled off by fibrosis and granulation tissue
Pancreatic pseudocyst
Contents of pancreatic pseudocyst
Fluid containing necrotic tissue and enzymes
Possible outcomes of a pancreatic pseudocyst
Spontaneous resolution
Compression of adjacent structures
Perforation
Benign tumor of the pancreatic tail resulting from inactivation of VHL gene. Gross morphology shows honeycomb of microcystic spaces.
Serous cystic neoplasm/cystadenoma of the pancreas
Microscopy of this pancreatic growth shows cystic spaces lined by glycogen-rich cuboidal cells that contain thin, clear, straw-colored fluid
Serous cystic neoplasm
Treatment for serous cystic neoplasm of the pancreas
Surgical resection is curative
Precursor lesion to invasive carcinoma of the pancreas
Mucinous cystic neoplasms
Genetic mutations associated with mucinous cystic neoplasms of the pancreas
KRAS mutation (50%)
RNF43 loss-of-function
TP53 and SMAD4
Gross appearance of larger cystic spaces filled with thick, tenacious mucin on the pancreas
Mucinous cystic neoplasms
Microscopy of mucinous cystic neoplasm of the pancreas
Cysts lined by mucin-producing epithelium
Dense, ovary-like stroma
Treatment of benign mucinous cystic neoplasm of the pancreas
Surgical resection is curative
Prognosis of invasive adenocarcinoma in mucinous cystic neoplasm of the pancreas
50% die within 5 yrs
Benign tumor of head of pancreas and major ducts that is seen in men. Can progress to invasive cancer.
Intraductal papillary mucinous neoplasm
Genetic mutations of intraductal papillary mucinous neoplasm of pancreas
KRAS (80%)
RNF43
GNAS
TP53 and SMAD4
Microscopy of pancreatic growth shows ducts filled with complex papillary projections lined by tall, columnar epithelial cells. Varying grades of dysplasia.
Intraductal papillary mucinous neoplasm of pancreas
Most common pancreatic carcinoma
Infiltrating ductal adenocarcinoma
Third leading cause of cancer deaths in the US
Pancreatic carcinoma
Most common precursor lesion of pancreatic carcinoma
Pancreatic intraepithelial neoplasia (PanIN)
Most common genetic mutation associated with pancreatic carcinoma
BRCA2
Syndrome associated with increased risk of pancreatic cancer
Peutz-Jeghers syndrome
Gross appearance of pancreatic ductal adenocarcinoma
Hard, stallate, gray-white, poorly defined mass
Microscopy of pancreatic tumor shows abortive tubular structures or cell clusters, and aggressive and deeply infiltrative growth pattern. Tends to grow along nerves and invade into blood vessels.
Pancreatic infiltrating ductal adenocarcinoma
Courvoisier sign
Palpably enlarged nontender gallbladder with mild painless jaundice
Associated with pancreatic carcinoma
Trousseau sign
Migratory thrombophlebitis caused by release of platelet-activating factors and procoagulants from tumor
Associated with pancreatic adenocarcinoma
Direct metastasis of pancreatic cancer tends to go where
Spleen
Adrenals
Transverse colon
Stomach
Primary areas of distant metastasis of pancreatic cancer
Liver
Lungs
Tumor markers associated with pancreatic cancer
CEA
CA19-9