Pancreatic, biliary tree, and salivary diseases Flashcards Preview

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Flashcards in Pancreatic, biliary tree, and salivary diseases Deck (79)
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1

Describe the normal pancreas and it's anatomical location

• Lobulated organ situated posterior to the stomach and anterior to the thoracic spine and ribs
• Retroperitoneal structure
• Traverses abdomen from left to right infero-diagonally, with the tail situated immediately medial to the splenic hilum and the head sandwiched within the C-loop of the duodenum
• Blood supply is dereived from branches of the superior pancreatoduodenal and splenic arteries off the celiac axis
• And Inferior pancreatoduodenal artery off the SMA
• Lies immediately anterior to SMV-PV confluence and SMA
• Immediately inferior/anterior to splenic artery and vein

2

What do acinar cells produce?

• A multitude of digestive pro-enzymes that are secreted across the apical cell membrane into a tiny ductule at the center of each acinus
• Ductules coalesce into the larger exocrine duct system of the pancreas that ultimately leads to the main (ventral) duct and ampulla of Vater
• In about 10% of people the dominant route of flow is dorsal duct and empties into minor papilla

3

Over 80% of the pancreas is what cell type?

• 80% of pancreateic cells are epithelial in origin and comprise the acinar glands
• Form the exocrine component and are of import in this first unit

4

What are the proteases secreted by the pancreas?

• Trypsinogen
• Chymotrypsinogen
• Proelastase
• Procarboxypeptidase A
• Procarboxypeptidase B

5

What are the 'other' enzymes secreted by the pancreas?

• Amylase
• Deoxyribonuclease
• Ribonuclease
• Procolipase
• Trypsin inhibitors
• Monitor peptide

6

What are the lipases secreted by the pancreas?

• Lipase
• Lipase-related proteins
• Prophospholipase A1, A2
• Nonspecific esterase

7

Why are the bicarbonate and water secretions of the pancreas important?

• They help move all the enzymes down to where they need to (flow is important, stasis is bad)
• They also provide a high pH environment to discourage the activation of the zymogens

8

What is going on in acute pancreatitis?


• Occurs when pancreatic enzymes are inappropriately and prematurely activated resulting in autolysis of the gland
• May result in severe inflammation and/or necrosis of pancreatic tissue
• Most commonly occurs when pancreatic duct becomes obstructed, resulting in stagnation of pancreas enzymes within the organ and activation of enzyme activation cascade

9

Why might alcohol intake precipitate pancreatitis?

• Direct toxic effect on pancreatic acinar cells
• Premature release and activation of trypsinogen and stagnant flow of pancreatic juice
*alcohol abuse will often result in pancreatitis (if it does) within 3-5 days of the binge

10

Some people do have premature enzyme activation, but what keeps this from being a problem normally?

• Peristalsis of duct
• Sphincer of oddi relaxation
• Bicarbonate and water secretion and flow
• Trypsin inhibitor function

11

What congential ductal abnormalities would you find obstructing the pancreatic outflow and thus being associated with pancreatitis?

• Pancreas divisum
• Annular pancreas
○ Usually these just increase the risk of alcohol use precipitating the event

12

Hyperlipidemia is associated with what pancreatic disease?

• Severe hyperlipidemia may precipitate acute pancreatitis for numerous reasons which remain poorly understood

13

What are the less common, but still testable causes of acute pancreatitis?

• Drug induced
○ Thiazide diruetics
○ Azathioprine
○ Anti-retroviral drugs
• Hypercalcemia
• Infectious
○ Mumps
○ Coxsackievirus
• Cystic fibrosis

14

What does the pancreas look like macroscopically and grossly in severe pancreatitis?

• Lipase released from dying acinar cells breaks down fat, liberating free fatty acids that precipitate with calcium ad form insoluble soaps
• Frank coagulation necrosis of the gland and/or hemorrhage into retroperitoneum
• Microscopically, necrosis of pancreatic tissue is associated with intense infiltrates of neutrophils and apoptosis of epithelial cells

15

What diagnostic tests are performed with clinical suspicion of pancreatitis?

• Blood test
○ Serum level of amylase and lipase
○ Elevated greater than 3 times the upper limit of normal
• Lipase more specific for pancreatitis and equally to slightly more sensitive than serum amylase
○ Rises 1-2 hours and decreases over following week
• Serum amylase rises and falls within 24-48 hours but its specificity is imperfect
○ Mumps, sjorgrens, penetrating peptic ulcer, intestinal trauma or ischemia, ectopic pregnancy
○ All these can confuse the findings

16

What imaging modalities can be used to help confirm the diagnosis of pancreatitis?

• Ultrasound and CT
• Ultrasound is cheaper and 90% accurate at detecting gallstones
○ BUT not great at looking at pancreas and ducts
• Contrast CT in severe cases or questionable situations
○ Shows inflammatory changes within and surrounding the pancreas-
§ gland edema, fat stranding, fluid accumulation
○ Can sometimes show the tumor cause
○ Certainly good for hemorrhage

17

What is ERCP?

Endoscopic retrograde cholangio-pancreatography (ERCP) is a diagnostic test to examine:

the duodenum (the first portion of the small intestine),
the papilla of Vater (a small nipple-like structure with openings leading to the bile ducts and the pancreatic duct),
the bile ducts, and
the gallbladder and the pancreatic duct.

18

How is acute pancreatitis treated?

• Hospital admission just in case things go south
• NPO status
• IV pain meds
• Time
○ Lame, yes, but majority of uncomplicated acute pancreatitis will improve with these supportive measures alone
• MUST avoid alcohol to prevent disease recurrence or progression to chronic pancreatitis
• Cholecystectomy later to remove source of obstruction
○ Otherwise healthy patients
• Stone removal by ERCP
• Can also be done surgically but that's more messy

19

Describe (general) the disease state of chronic pancreatitis

• Condition that develops after repeated bouts of acute pancreatitis
• Commonly occurs as a result of chronic alcohol abuse
• Macrospic
○ Characterized by replacement of healthy pancreatic tissue by hard fibrous tissue
○ There may be atrophy of the gland as well
• Pancrease juice may become viscous, and calcifications/stones may devleop within duct if these clumps of protein precipitate with calcium salts
• Microscopic
○ Broad bands of scar tissue replace lost lobular tissue
○ Can show presence of lymphocytes and plasma cells
• There is usually sparing of islet cells
• Fibrous tissue can cause strictures of duct
• Calcified stones can precipitate obstruction
• Malabsorption, pain, malnutrition

20

What is the most common cause of CP in the west?

• CP = chronic pancreatitis
• Chronic alcohol abuse
• Cigarette smoking also contributed to fibrosis, and particularly in alcoholics

21

What are the inherited conditions that predispose people to chronic pancreatitis?

• CFTR mutations
• Trypsinogen gene mutations
○ PRSS
• Trypsin inhibitor mutations
○ SPINK
• Familial hypertriglyceridemia
• Equatorial countries - idiopathic variant that has extensive calcifications, called tropical pancreatitis

22

Why do pancreatic stellate cells have an important role in chronic pancreatitis?

• They, when stimulated, proliferate and transform into collagen-synthesizing cells
• Can contribute to the ductal obstruction

23

Fat malabsorption is a marker of what degree of pancreatic damage?

• 90% of the organ is destroyed before fat malabsorption is seen
• 95% for carbs and proteins because trypsin and amylase are produced elsewhere as well

24

What are the clinical manifestations of chronic pancreatitis?

• Steatorrhea is important finding
• Epigastic pain that radiates directly to back
• Can just present as back pain alone
• Pain will wax and wane but never truly disappear

25

What is steatorrhea

• Fatty diarrhea
• Frequent, oily, foul-smelling, and/or buoyant stools
• Increased flatulence and weight loss

26

What might patients with chronic pancreatitis have B12 deficiency?

• Pancreatic proteases are required to cleave the R-protien-cobalamin complex
• This allows for intrinsic factor to bond to B12
• Thus, pancreatic duct obstruction or atrophy will result in a loss of B12 binding to IF, and thus a malabsorption of B12
• Eventually causes macrocytic anemia

27

Why might bleeding diathesis develop in chronic pancreatitis?

• A result of fat-soluble vitamin malabsorption, specifically vitamin K

28

What diagnostic tests for chronic pancreatitis are available?

• History and physical can be highly suggestive
• Plain x-ray of abdomen can show calcifications scattered over epigastrium in severe calcific disease
• Rapid fat stool stain
○ Sudan stain, qualitiative
• Definitive is 72-hour quantitative stool collection for fat analysis
○ High fat diet of over 100g of fat/day
○ 72 hour stool collection should who over 10-20% fecal fat excretion or 50 g of fat

29

Pancreatic cancer is a major US killer. From what cells does this cancer arrive?

• Vast majority (90-95%) arise from ductal epithelial cells
• Remaining come from acinar cells
• Both carry a bad prognosis
• Cancer usually forms primitive, mucin-positive, gland-like structures
• Cells elicit a strong, fibrotic reaction known as desmoplasia, texture very hard and cancer cells less permeable to chemotherapy drugs
• Often have microscopic tumor tendrils that are not seen by imaging

30

What are the much more rare pancreatic tumors?

• Mucinous cystadenocarcinomas
• Intraductal papillary mucinous tumors
○ IPMTs
• Arise from cystic lesions in pancreas and much less common than ductal adenocarcinoma
• More favorable prognosis

31

What are the risk factors for pancreatic adenocarcinoma?

• Positive family history
• Tobacco abuse
• Chronic pancreatitis
• Obesity
• Ocassionally a genetic syndorme
○ Peutz-Jeghers
○ Von Hippel-Lindau

32

What is tousseau's syndrome?

• Associated with pancreatic adenocarcinoma
• Hypercoagulable state associated with neoplasm and decrease in physical activity

33

What imaging studies can result in dx of pancreatic adenocarcinoma?

• Contrast abdominal CT scan
○ 80-90% sensitivity
○ Shows pancreas, bile ducts and pancreatic duct very well
○ Also decent for detecting distant metastasis
• Ultrasound
○ Less accurate because of fat and air surrounding pancreas

34

What imaging studies can result in dx of pancreatic adenocarcinoma?

• Contrast abdominal CT scan
○ 80-90% sensitivity
○ Shows pancreas, bile ducts and pancreatic duct very well
○ Also decent for detecting distant metastasis
• Ultrasound
○ Less accurate because of fat and air surrounding pancreas
• Biopsy or fine-needle aspiration
○ FNA = fine needle aspiration
• Endoscopic ultrasound is the up and coming modality
• Also helps with pre-op tumor staging as it shows borders and lymph nodes in the region very well

35

Insulinomas, glucoagonomas, gastrinomas, VIPomas and somatistatinomas all have what in common?

• They are neuroendocrine tumors that secrete a given hormone in excess that forms a particular clinical picture
*found by imaging and by fine needle aspiration

36

What might glucoagonomas cause?

• Hyperglycemia
• Deiabetes
• Weight loss
• Diarrhea

37

What might somatistatinomas cause?

• These are rare
• Weight loss
• Malabsorption
• Acalculous cholecystitis
○ Decreasing GI motility and exocrine secretion

38

What might VIPomas cause?

• Severe, chronic, secretory type diarrhea with hypokalemia and weight loss

39

What might gastrinomas cause?

• GERD or peptic ulcers
• Diarrhea
• Fat malabsorption

40

Why is surgery recommended for anybody with an NET that can handle the surgery?

• NET = neuroendocrine tumor
• These cannot be graded/staged for metastatic nature by histology
• You can only watch them as they invade
• Thus, get them out. Not all have the worst metastatic abilities, but glucagonoma and somatistatinoma are almost always malignant

41

How do you treat autoimmune pancreatitis?

• 6 week course of PO corticosteroids
○ Prompt and effective
• Jaundice and pruritis (itchy) patients may benefit from ERCP with placement of a temporary biliary stent.
• Immunomodulators can be used…but that's a case by case basis and in refractory cases

42

What studies are done to show autimmune pancreatitis?

• Serum IgG-4 is elevated in about 80% of patients
• Imaging shows an enlarged pancreas with decreased enhancement and loss of the lobular contour
• ERCP will show narrowed pancreatic duct and sometimes a bile duct stricture
• EUS-guided or percutaneous biopsy of the pancreas and IgG-4 staining can be definitive dx modality

43

What does a patient with AIP usually look like?

• NOT your typical chronic pancreatitis patient
• No alcohol abuse usually
• 45-70 year old males
• No history of pancreatitis at all, not even acute
• Present with chronic epigastric or diffuse abdominal pain and nearly half also develop cholestasis
○ Jaundice, dark urine, itching
• Associated with other auto-inflammatory diseases like RA, IBS, lupus or sjorgren's syndrome

44

What immune-associated factors and cells are implicated in the disease process of autoimmune pancreatitis?

• AIP = autoimmune pancreatitis
• IgG-4 and plasma cells and lymphocytes infiltrate the pancreas and its vessels
• Results in localized or diffuse enlargement of pancreatic parenchyma and narrowing of pancreatic duct and/or bile duct
• YOU WILL NOT FIND
○ Glandular atrophy, ductal dilation, calcifications and steatorrhea are features of chonic pancreatitis but NOT features of AIP

45

What does the gallbladder normally do?

• Storage place for bile, so that it can be released in higher amounts when the diet calls for it
• Bile is a yellow liquid with amphopathic properties that contributes to excretion of various compounds
○ Cholesterol, copper, medications
• Also helps with lipid digestion within the small bowel
• Contains water, bile acids, cholesterol, phospholipids, lecithin, electrolytes
• Bile acids are main active ingredient in bile and contribute the most to lipid digestion

46

What are the constituents of bile?

• cholesterol, phospholipids, lecithin, electrolytes
• Bile acids are main active ingredient in bile and contribute the most to lipid digestion

47

Describe the pathway of bile formation and movement into the gall bladder

• Made by hepatocytes and secreted into the cannaliculi
• Drain into peripheral intrahepatic bile ducts
• Intrahepatic bile ducts coalesce into the right and left hepatic ducts, which fuse to form the common hepatic duct

48

Describe the gall bladder during both fasting and fed states.


• Fasting
○ Vagal tone and CCK levels are decreased
○ Sphincter of Oddi is closed
○ Gallbladder and bile duct peristalsis are inhibited
○ Secretions of bile from liver hit the sphincter and retrograde flux into gallbladder for storage
• Fed
○ Vagal tone and CCK levels are increased
○ Sphincter of Oddi is openend and the gall bladder and bile duct peristaltic movements are increased
○ Results in flow from gallbladder into the duodenum through cystic duct

49

What happens to the bile while it sits in the gallbladder?

• There is constant transport of sodium from the lumen of the gallbladder into the bloodstream
• Water will follow this passively
• End result here is concentration of the bile

50

What are the risk factors for generation of cholesterol stones?

• Obesity
• Rapid weight gain or loss
• Female gender
• Age over 30
• Latin American or Native American ethnicity
• Estrogen/contraceptive use

51

What is the process behind gallstone formation?

• There has to be supersaturation
○ Can be from too little water or too much cholesterol
○ Could be both processes as well
• Supersaturation of bile with cholesterol first results in formation of cholesterol crystals
○ Microlithiasis
• Eventually clinically significant stones can develop
• Numerous factors contribute:
○ Stasis in gallbladder or bile duct, hereditary mutations in cholesterol chain structure, inflammation of gallbladder

52

What are the different types of gallstones?

• 3 different types
○ Cholesterol, brown and pigment
• Cholesterol stones
○ Cholesterol, bile acids, phospholipids and lecithin
○ White or yellow, soft and greasy
• Pigment stones
○ More common in asians
○ Predominantly of calcium bilirubinate salts which coalesce around a mucin nidus
○ Come from increased concentrations of bilirubin in bile
§ Hemolytic states like sickle cell anemia
○ Can develop in gallbladder OR straight into cystic duct
○ Think about the parastic infections like clonorchis that can result in cholangitis and stasis
§ Endemic in Asia so these stones are more common in asia
• Brown stones
○ Hybrid of cholesterol and pigment stone crystals
○ Develop de novo within bile duct as a result of infection in patients with prostheses
§ Tubes or stents
○ Can develop due to a downstream obstruction

53

What is the best diagnostic study (at least initially) for gallstones?

• Abdominal ultrasound
• Over 90% accurate for gallbladder stones or cholecystitis
• Good for seeing bile duct diameter, and possible ductal dilation
• Not as good for seeing stones in the bile duct itself

54

How might you treat a bile duct obstruction?

• ERCP
• Retrograde cholangiopancreatography
• Essentially a scope, and they can protrude needles from it for biopsies or minor surgeries
• Can remove stones or place stents during procedure
• Less invasive
○ Potential risk of pancreatitis

55

What is biliary colic?

• Occludes the gallbladder intermittently
• Usually when patient consumes a meal (particularly fatty meal b/c of CCK release)
• Dull or crampy pain in the epigastrium or upper right upper quadrant which occurs within an hour of eating and resolves spontaneously
○ Typically within 3-5 hours
○ Has to do with time for hormone to go down or stone to move

56

What is acute cholecystitis?

• Stone has compacted the bile duct and now there is a bacterial superinfection of gallbladder
• Severe inflammation and/or ischemia of the gallbladder, usually associated with infection
• Symptoms
○ Severe RUQ pain
○ Radiation to right flank or shoulder
○ Focal tenderness to deep palpation of the RUQ and palpation during expiration
○ Murphy's sign - strongly suggestive of cholecystitis

57

What is the treatment paradigm for acute cholecystitis

• Similar to acute pancreatitis
• Hospitalization for pain, but not much else
• IV fluids
• NPO for gallbladder rest
• IV antibiotics (is this controversial too?)
• Pain mediations
○ Cholecystectomy is treatment of choice for all surgical candidates within 1-2 weeks of event

58

What serum tests will be affected by acute cholecystitis?

• Neither cholecystitis or gallbladder stones will significantly elevate liver enzymes
• Exception - Mirizzi's syndrome
○ Stone lodged in cystic duct causes severe, localized edema and inflammation to develop in the region
○ Leads to benign obstructino of common bile duct or common hepatic duct
○ The other way this can happen is a very fat and enflamed gallbladder physically pushing on the ducts and obstructing them
• Treatment is cholecystectomy

59

What is acalculous cholecystitis?

• Similar to stone-caused or calculous cholecystitis, but it's an ischemic process not an obstructive process
• Vasculitis (polyarteritis nodosa)
• Hypoperfusion event
○ DIC, sepsis, MI, burns, severe trauma and blood loss, etc.

60

What does choledocholithiasis mean?



• Gallstones migrating past cystic duct into common bile duct
• If they get lodged in or before the ampulla of vater they can cause
○ Ascending cholangitis and/or acute pancreatitis
What does RUQ pain, jaundice and dark urine suggest to you?
• This constellation of symptoms looks like choledocholithiasis

61

What is the treatment for ascending cholangitis?

• Remember charcot's triad and reynold's pentad
• Admission to hospital and IV antibiotics
• Urgent ERCP with stone extraction or stent replacement is necessary often

62

Chorcot's triad can progress to Reynold's pentad which is…?

• Triad + hypotension and confusion
• Fever
• Pain
• Jaundice
• Hypotension
• Confusion

63

What is Charcot's triad?

• Indicative of ascending cholangitis
• RUQ pain
• Jaundice
• Fever

64

What is up with gallbladder cancer?

• Adenocarcinoma is the only one we talked about
• Risk factors are gallstones and chronic cholecystitis
• Infection with liver flukes (parasites) may also cause recurrent cholecystitis and chronic cholecystitis enough to present a risk factor
• Poor survival rate as this is usually caught pretty late in the disease process
• If cancer causes obstruction it can be diagnosed sooner on cholecystectomy
• Gallblader cancers are also similar to pancreatic carcinomas as they produce thick stroma connective tissue

65

What is primary sclerosing chlangitis?

• PSC = primary sclerosing cholangitis
• Remember UC predisposes you to this
• Predominantly affects caucasian males 30-60
• Gallbladder is usually spared
• Lots of strictures through the biliary tree
• Higher risk of developing cholangiocarcinoma

66

What are gray/white colored stools called?

• Acholic
• Absence of bilirubin in the stools

67

In a patient that has already had a cholecystectomy and they are still having symptoms of biliary colic, what are you thinking?

Sphincter of Oddi problems
*there can be a dysfunction of the sphincter of oddi that keeps it spasming and not allowing bile to flow through
*can cause ascending cholangitis in really bad cases, even in a patient with absolutely no way to form gall stones

68

What are the 8 salivary gland diseases covered in our notes?

• Mumps
• CMV sialadenitis
• Bacterial Sialadenitis
• Sarcoidosis
• Sjorgren's Syndrome
• Salivary Lymphoepithelial Lesion
• Xerostomia (dry mouth)
• Halitosis

69

Because salivary glands are ductal tissue, what disease are they predisposed to?

• Ductal neopslams
• Adenoma or adenocarcinoma

70

What is pleomorphic adenoma?

• Salivary neoplasm discussion here
• Diverse microscopic pattern is characteristic
• Islands of cuboidal cells arranged in ductlike structures is a common finding
• Loose chondromyxoid stroma, connective tissue, cartilage and even osseous tissue
○ Thus pleomorphic
• Typically encapsulated
○ Tumor islands may be found within fibrous capsule

71

What's up with Warthin's tumor?

• Benign papillary cystadenoma lymphomatosum
• Second most common benign tumor of parotid gland
• 2-10% of all parotid gland tumors
• Bilateral in 10% of cases
• Mucoid brown fluid in FNA (fine needle aspiration)

72

What two components need be present to diagnose warthin's tumor?

• Lymphoid and epithelial components
• Epithelial - papillary fronds with two layers of oncocytic epithelial cells
○ Cytoplasm pink b/c lots of mitochondria
○ Occasionally looks like squamous metaplasia, and often confused for SCC
• Lymphoid component
○ Occasional germinal centers are seen
○ Lymphoid tissue forms core or papillary structures
○ This is more present than the epithelial or oncocytic component

73

Besides pleomorphic adenoma, what else can be found in the mouth?

• Monomorphic adenoma, usually salivary gland presence
• Composed of uniform basaloid epithelial cells with monomorphous pattern
• Absence of the many different types of stromal cells
• Much more uniform in appearance, ducts surrounded by stroma
*important because they have a malignant potential. Still not majority, but something to be aware of

74

What are the malignant neoplasms associated with salivary glands in order of frequency?

• Mucoepidermoid carcinoma
• Polymorphous low grade adenocarcinoma
• Adenoid cystic carcinoma
• Clear cell carcinoma
• Acinic cell carcinoma (most common)
○ No glycogen, fat and mucin
○ 3% malignant and bilateral)

75

What is the most common malignant neoplasm with the salivary gland?

• Acinic cell carcinoma (most common)
○ No glycogen, fat and mucin
○ 3% malignant and bilateral)

76

A patient has a bump around where the salivary glands are. What items in their history and physical would make you concerned for malignancy?

• Induration/hardness of lump
• Fixed to overlying skin or mucosa
• Ulceration of skin or mucosa
• Rapid growth
• Short duration
• Pain (usually out of proportion)
• Facial nerve palsy

77

Swiss cheese pattern is a buzz word for what condition?

• Adenoid cystic carcinoma
• Salivary gland neoplsm
• Second most common salivary gland neoplasm
• Submandibular, sublingual and minor glands

78

you see on histology a gland filled with a thick substance. What are you thinking?

Mucoid Adenocarcinoma.
*either high or low grade depending on how well circumscribed the tumor is

79

What are the exocrine pancreatic cancers in descending order of frequency that we discussed?

• Adenocarcinoma (ductal)
○ 90% of all pancreatic cancer starts in the duct
• Adenosquamous carcinoma
○ Glands flatten out as they grow
• Intraductal papillary-mucinous neoplasm
○ Finger-like projections into duct
○ Prelude to malignancy
• RARE
○ Mucinous cystadenocarcinoma
§ Spongy cystic tumor
○ Pancreatoblastoma
§ KIDS, suspect in infancy
○ Acinar cell carcinoma
§ Look for too much lipase chronically due to overproduction by tumor