Lecture 74_75 - Diseases and pathology of Pancreas, GB Flashcards
Describe the anatomy of the biliary tree?
Right Hepatic duct and left hepatic duct converge to form the common hepatic duct (RHD branches more quickly than the left)
Cystic Duct (from GB) converges with the CHD to form the Common bile duct
Pancreatic duct and CBD converge at the Major Papilla (of Vater) (of the second part of the Duodenum), which is controlled by the Sphicter of Oddi
what is ERCP?
Endoscopic Retrograde Cholangeo-pancrea-tography
Imaging technique designed to specifically look at biliary architecture
what is a HIDA scan?
§ HIDA Scan – aka Liver/Spleen Scan
□ Radio tracer injected into the vein which follows the course of bile (taken up in liver and excreted into biliary tree)
® Liver should be dark; excreted to bile duct
® If stone in the Cystic Duct – no tracer will enter the gall bladder
what are the different types of gall stones?
etiologies
1) Cholesterol stones – Comes from endogenous cholesterol when there is an imbalance between cholesterol, bile salts, and phospholipids, the cholesterol comes out of solution
2) Pigment Stones –
black – increased unconjugated bile (such as hemolysis of sickle cell disease)
brown – usually due to infection; common in asian populations (liver flukes)
Treatment of Gall stones;
Symptomatic
asymptomatic
Asymptomatic – observation; unless the patient needs prophylaxis (eg sickle cell patients or porcelien GB patients)
Symptomatic – Threapuetic intervention is necessary due to increase complication rate
Symptoms of Gall STones / impaction of a gall stones
(where can the pain radiate to?)
what symptoms should you not attribute to gall stones?
Symptoms: Biliary Cholic (episodic and crampy pain due to a stones transient impaction in the cystic duct)
pain is usually in the RUQ/Epigastrium but can radiate to the chest or R scapula
Never attribute Chronic pain, belching, bloating, fatty food intolerance or chronic pain to gall stones
Pirmary method for dx of gall stones?
Ultrasound
Complications of cholelithiasis?
what pattern of LFTs is observed
- Acute cholecystitis –
- Choledocholithiasis – (Choledocholithiasis Cholangitis vs Choledocholithiasis Pancreatitis)
LFTs- – elevated BR / elevated ALP
• Acute cholecystitis –
where is the stone?
typical presentation
eponymous physical exam finding
diagnostic techniques
Treatment
gall stone stuck in the cystic duct, leading to inflammation of the GB wall, due to bacteria in the now static media
resentation – RUQ pain with N/V, Fever, Luekocytosis, Jaundice,
Murphy’s Sign (palpate the gall bladder and have inspiratory arrests)
dx: RUQ pain, US, HIDA
Tx: Abx + surgical management
Choledocholithiasis Cholangitis — where is the stone
what is charcot’s triad?
Choledocholithiasis Pancreatitis
Cholangitis – § Common bile Duct Obstruction leading to bacterial infection and inflammation
§ Charcot's Tirad -- pain, jaundice, fever
Pancreatitis – Inflammation of the pancreas due to gallstone obstruction in the pacnreatic duct/ampulla of vater
Treatments for choliolithiasis
○ Surgery – laproscopic cholecystectomy
Oral dissolution – bile salts over many month
Contact Dissolution — infuse ether , and solubilizes stones instantly, but lots of side effects
○ Extracorporial lithotripsy
ERCP – § Preferred method for removal of common bile duct stones
• Primary Sclerosisng Cholangitis
what is it?
histo pattner?
commonly associated with?
how does it present on imaging?
Idiopathic fibrosing d/o of the intra and extra hepatic biliary ducts
○ Associated with IBD (Crohns, Ulcerative Colitis)
onion skinning of bile ducts
imaging: “chain of links”
what is acute pancreatitis?
what is the a necessary symptom to make the dx?
Acute inflammation of the pancreases manifested by abdominal pain and increased amylase and lipase (atuodigestion of the pancreas by pancreatic enzymes)
PAIN is an absolutely necessary symptom to make the dx
what kind of pain can manifest in acute pancreatitis?
what levels of amylase and lipase are seen on labs?
○ PAIN – epigastric, central and radiates to the back (bc the pancreas is retroperionteal)
§ Some of the worst pain a patient can have
○ Enzymes Amlyase and Lipase – 3x upper limits of normal
what are some etiologies of acute pancreatitis (GET SMASHED)
Gall-stones Alcohol Trauma Steroids/sulfonamides, other durgs such as 6MP Mumps Auto-immune disease Scorpion sting Hypercalcemia/Hypertriglyceridemia ERCP Drugs (sulfa drugs)
what are some effcts (local and systemic) of increased trypsin and lipase ?
○ Activation of multiple cascades
§ Capillary leak
§ Peripheral white cell response
§ DIC
§ Local Fat Necrosis - -autodigestion of the pancrease
§ Coagulopathy
Renal failure
can lead to pancreatic psuedocyst which can rupture and hemorrhage
Complications of acute pancreatitis?
how may it present
Psuedocysts – Extrapancreatic Fluid in the retroperitoneal space; a rind can form around it
§ Pseudocysts – form 3-6 weeks after sever acute pancreatitis
Severe pain, obstruction duodenum, dissection, bleeding of the cyst (hemosucus pancreaticus), infection, erosion into other structures, pleural effusion with high amylase and lipase
Treatment goals of Chronic pancreatitis
Supportive care – clinical surveillance, NPO, aggressive IV fluid replacement (helps perfuse the pancreas and mitigates renal dysfunction), pain relief , nutritional support , (+/- IV abx of necrosis)
○ Reduce inflammation ○ Assess and treatment complications
• Chronic Pancreatitis –
What is it ?
• Presentation ?
Long standing inflammation in abnormal gland, usually after repeat bouts of acute pancreatis which has caused gland scarring
○ Chronic pain ○ Calcificaitons of the pancrease ○ Pancreating endo and exocrine insufficiency
Major etiologies of chronic pancreatitis?
Alcohol's CF, herediatary pancreatitis, hypertriglyceridemia autoimmune Idiopathic
Pancreas Divisum
Presentation and symptoms of chronic pancreatitis?
Pain (which may die down as the disease progresses)
exocrine insufficienies
Steatorrhea (if alcohol related) (lipase < 10%)
endocrine insufficiencies:
DMT1 – loss of insulin and glucagon
Risk of DKA
what dx test can be made for chronic pancreatitis?
ERCP, CT, US – look for stones, ductal dilation
□ Calcifications in the gland —
§ Function -- Secretin tests (enzymes, bicarb), fecal chymotrypsin, or fat
- acute cholecystitis vs chronic
Gross path
symptoms
acute: GB appears red or hemorrhagic
RUQ pain, N, Fever, anorexia
Chronic:
The GB wall is thick and fibrotic
RUQ Pain, N, Fever, anorexia — but usually a more indolent course
May be asymptomatic
Biliary Atresia:
what is it?
etiology?
pathology?
prognosis?
Hypoplasia or narrowing of biliary tract in infancy;
Pathology — Cholestasis; bile tries to found other routes of the liver; biliary cirrhosis; bile duct obliterated by inflammation
* Etiology unknown * Most important cause of liver disease in early childhood -- frequent and severe
Prognosis – ○ Cirrhosis develops by 3-6 months (8 weeks) of age without treatment
biliary atresia – treatment
complications of the treatment
Kasai Procedure – transection of the small bowel such that the bile ducts can drain directly into the bowel
§ Complications: Increased Incidence of Cholangitis § Many patients go on to develop cirrhosis later in life anyway
Choledochal Cyst
- what is it ?
how does it present?
treatmnet
- Congenital, tpyically present with obstructive jaundice
- - Treatment: Resection
three Congenital Abnormalities of the Pancreas
Pancreas Divisum — small Duct of Wirsung; most of the pancreas is left to the drain through minor papillum.
Pancreatinc Heteropia ( normal pancreatic tissue elsewhere in the GI tract)
Annular Pancreas – a pancreas that encircles the duodenum;
Predispose: to Pancreatitis
Acute vs chronic pancreatitis
pathologies
histology –
(what happens to the ducts in chronic pancreatitis)
Acute:
Pathology: Inflammation, fat necrosis, Hemorrhage
Release of Digestive Enzymes (lipases, phospholipase, elastase)
Local and Distant Injury
Histology — viable pancreas, necrosis (no nuceli, red is dead), fat necrosis (white lobules)
Chronic:
loss of excorine and then endocrine function
patients present with steatorrhea, then DM symptoms
first – loss of the acini
second – loss of the islets
replacement by fibrosis
dilation of the ducts
Pancreatic Cysts:
what are the different kinds
which are benign vs have malignant potential vs malignant
Non neoplastic/Benign: Congeital, Psuedocysts
Neoplastic/Benign: Serous Cysts
Neoplastic/Malignant Potential: Mucinous cysts (MCNs, IPMNs)
neoplastic/malignant: Solid pseudopapillary + cystic changes in actual tumors
treatment options for cysts?
Watch
Cytology (FNA) to risk stratification
Excision
Pseudocysts
benign vs malignant?
associated with?
why is it called psudocyst?
Prognosis?
non neoplastic; Benign
Usually in the setting of chronic pancreatits (alcohol)
but can result from inflammation or trauma
lack true lining
prognosis: May resolve, become infected (in the pool of protein), or impinge on other structures
Serous Cysts -
benign vs malignant?
Gross path appearance?
males vs females predominance?
Prognosis?
Neoplastic; Benign
○ ‘Sponge-like’ gross appearance
○ Female predominance (2:1), older adults (7th decade)
○ May become very large, present as mass
Mucinous Cysts
benign vs malingnant?
what are the two kinds?
Potential for Malignancy
○ Mucinous Cystic Neoplasm: MCM ○ Intraductal Papillary Mucinous Neoplasms -- IPMN
Mucinous Cysts: Intraductal Papillary Mucinous Neoplasms – IPMN
- male or female predominance?
- where in the pancreas does it occur?
§ Older patients, male predominance, more in the pancreatic head
Mucinous Cyst: Mucinous Cystic Neoplasm: MCN
- male or female predominance?
- where in the pancreas does it occur?
- histology appears like what other organ?
§ Young women, in body or tail of pancreas
§ ‘ovarian type’ stroma
Pancreatic Cysts: Solid Pseudopapillary Tumor
benign vs malingnant?
- male or female predominance?
what mutations/proteins are good for diagnosis ?
treatment and prognosis of treatment?
Malignant but very low-grade
Strong female predominance (9:1), young patients
Mutations in the Beta-catenin/APC
○ Treatment: Resection is curative
Pancreatic Cancers:
vast majority are….?
precursors include …?
genetic pathogenesis…(name a few genes which become mutated?)
Vast Majority: Ductal Adenocarcinoma
Development of Pancreatic cancer: Cystic Lesions (mucinous -- MCMs, IPMNs) --- may progress to cancer (benign --> low grade --> high grade --> invasion)
Mutations In K ras
Inactivation of P16
Inactivation of P53
SMAD4 = DPC4 = ("deleted in pancreatic cancer")
Ductal Adenocarcinoma –
mortality?
reason for this mortality?
where in the pancreas are the typically found?
histology?
High mortality — 7% survival at 5 year
Presents at high stage bc you have to wait for symptoms. No effective screening methods
Majority (60%) are in the Head
Histology -- Invasive gland like structures Perineural invasion Invasion outside of pancreas Lymphatic and vascular invasion
Treatment options for pancreatic malignancies:
what are the different types of surgical procedures
what can be done beside surgery
Surgical - the only real treatment
Head of Pancreas: Whipple Procedure (pancreatic duodenectomy)
Body/Tail of Pancreas: Distal pancreatectomy & Splenectomy Neoadjuvant therapy may be given in unresectable cases to possible shrink the tumor enough to make it resectable Adjuvant Radiation and Chemotherapy
Pancreatic Endocrine (Islets) Neoplasms
what stains can be done on histology?
what are they histological classification? — which can be treated with chemo?
what are the functional classifications? — which are benign / malignant?
stain positive for chromogranin and synaptophysin
Histo class:
§ Well differentiated tumor (type 1) – not treated with chemo
§ Well differentiated carcinoma (type 2) — treatment with chemo
Poorly-differentiated carcinoma (type 3; rare)
○ Functional classification § Insulinomas: Majority are benign (symptomatic at early stage) § Gastrinomas: Majority are malignant Glucagonomas: Majority are malignant
Acinar Cell Carcinoma of the pancreas
- characteristic feature?
Arises from acinar cells rather than ducts
§ Increased levels of amylase and lipase
Pancreoblastoma
- who is mostly affected by this?
how common is it?
histology?
treatment
Rare – mainly in children
Histology: Undifferentiated cells with squamoid nests Treatment: Chemotherapy
