Periampullary Tumor

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Pancreatic and peri-ampullary CA  Ductal adenoCA  most common primary malignant disease of pancreas and peri- ampullary region  Accounts for more t han 75% of all non- endocrine tumors in this region Ranks 11 th among all cancers

Transcript of Periampullary Tumor

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Pancreatic and peri-ampullary CA 

Ductal adenoCA – most common primarymalignant disease of pancreas and peri-ampullary region

 Accounts for more than 75% of all non-endocrine tumors in this region

Ranks 11th among all cancers

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Most lethal CA – overall 5-yr survival rate <3%

5th leading cause of cancer death

 Ampullary, distal CBD, duodenal adenoCA – less common than pancreatic tumor; accountsfor 15-20% of all peri-ampullary malignantdisease.

Pancreatic and peri-ampullary CA 

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Pancreatic and peri-ampullary CA 

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Peri-ampullary CA (ampulla, distal CBD,duodenum) – associated with increased age,HNPCC, Peutz-Jeghers syndrome, familialadenomatous polyposis, Gardner’s syndrome.

Pancreatic and peri-ampullary CA 

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Pancreatic and peri-ampullary CA 

Can be broadly classified as primary, metastatic, orsystemic

Primary cancers can demonstrate either endocrine or

nonendocrine differentiation Most common malignant diseases that metastasize to

the pancreas are renal cell, breast, colorectal, small celllung and melanoma

Systemic malignant conditions involving the pancreasinclude leukemia and lymphoma

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Ductal AdenoCA (1o solid non-endocrineepithelial tumor)

By far the most common peri-ampullary malignant

disease

 Typically aggressive; most resected adenoCA havealready metastasize to regional lymph nodes

Papillary ductal lesion are 3x more common inpatients with pancreatic CA than in normal pancreas

Display k-ras ,  p53,  p16 mutation

Pancreatic and peri-ampullary CA 

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 Adenosquamous CA –  variant of adenoCA; occursin patient’s with history of chemoradiation

Giant cell CA – accounts for <5% of solid

pancreatic malignant tumors Acinar cell CA – distinct histologic appearance

(often greater than 10cm)

Pancreatoblastoma – or pancreatic cancer of infancy;occur in children up to the age of 15yrs.

Pancreatic and peri-ampullary CA 

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 Vague symptoms early the course

Develop obstructive jaundice secondary toobstruction of the intrapancreatic portion of theCBD

 Jaundice often associated with pruritus, acholicstools and dark urine

Pain described as vague upper abdominal,epigastric or back discomfort

Clinical Presentation

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Staging

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Non-operative palliation Most peri-ampullary adenoCA have unresectable

tumors at presentation

Palliation is aimed at three major symptoms:obstructive jaundice, duodenal or gastric outletobstruction and tumor-associated pain

Patients found to have distant metastases,unresectable local disease, or disseminated intra-abdominal tumors are appropriate candidates fornon-operative therapy 

 Age alone is not a contraindication to surgicalresection or palliation

Palliative Therapy

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Biliary obstruction Biliary decompression can be achieved by either

endoscopic or percutaneous transhepatic technique

PBD first reported in 1974; catheter exchanges doneevery 3mos to prevent cholangitis and recurrentjaundice secondary to stent occlusion

Endoscopic approach is the method of choice

because of lower procedure-related morbidity andmortality; removal and replacement of stent every 3-6mos to prevent recurrent jaundice and cholangitis

Palliative Therapy

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Palliative Therapy

Pain Pain associated with pancreatic cancer is unremitting and

often poorly managed

Postulated causes include tumor infiltration into the celiacplexus, pain associated with early satiety, gastroduodenal

obstruction and gallbladder or biliary obstruction, increasedparenchymal pressure caused by pancreatic duct obstruction,and pancreatic inflammation

 Tumor-associated pain is best treated with long-acting oralanalgesic in appropriate dosage, pain management specialistmay be required to help manage this problem

For pain intractable to typical narcotic regimens, CT guidedpercutaneous celiac nerve block, external beam radiationtherapy, and thoracoscopic or endoscopic chemical

splanchnicectomy maybe done.

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Palliative Therapy

Duodenal obstruction Surgical gastrojejunostomy or feeding tube insertion

maybe done

Endoluminal approaches with biliary-typeexpandable metallic stents are now being tested

Operative palliation Tertiary centers report resectability rates for peri-

ampullary cancers ranging from 67-89%

Palliative surgery is indicated in patients whosetumors are found to be unresectable at the time oflaparotomy intended for curative resection

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Operative Palliation

Obstructive jaundice

Most commonly performed surgical procedure for relief ofobstructive jaundice include hepaticojejunostomy or

choledochojejunostomy, choledochoduodenostomy andcholecystojejunostomy 

Pain

Chemical splanchnicectomy can be performed to alleviate the

debilitating pain at the time of operative palliation; performedby injecting 20ml of 50% alcohol through a spinal needle oneither side of the aorta at the level of celiac plexus

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Resectional Therapy

Pancreaticoduodenectomy for peri-ampullary tumors

Halsted performed the first successful resection of a peri-ampullary tumor in 1898

Kausch performed the first successful en bloc resection usingtwo-stage approach

 Whipple and colleagues popularized the procedure in the1930s and 1940s

Pylorus-preserving pancreaticoduodenectomy is now thefovored procedure because the gastric reservoir and thepyloric mechanism are kept intact

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Resectional Therapy

Operative technique

If the tumor is localized to the periampullary regionor head, neck, or uncinate process of the pancreas,

 with no evidence of distant metastatic disease of thepancreas or major vascular involvement, the surgeonproceeds with resection

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Complications Operative mortality rate for pancreaticoduodenectomy is

currently less than 3% in centers specializing in pancreaticsurgery 

Despite this rate, incidence of post-operative complicationsremains as high as 40-50%  Two leading causes of morbidity are early delayed gastric

emptying and disruption or leak at the pancreatic anastomosis(pancreatic fistula); the cause is likely multifactorial

Erythromycin has been shown to improve gastric emptyingafter surgery  By definition, pancreatic fistula occurs 7 or more days post-

op, when the drain output contains milky, amylase-rich fluidin excess of 50ml/day 

Resectional Therapy

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Prognostic Factors

Prognostic factors Determined by multiple factors including tumor stage,

biologic features, molecular genetics, post-op factors and theuse of adjuvant chemoradiation

Peri-ampullary tumors including distal bile duct, ampullary,and duodenal adenoCA are less common, overall 5-yrsurvival rate is better

In long term survivors with resected peri-ampullaryadenoCA, the site specific 5-yr actual survival rates were 15%

for pancreatic CA, 27% for distal bile duct CA, 39% forampullary CA, and 59% for duodenal CA  Well-differentiated tumors, negative resection margins, and

negative nodal status were indicators of a better prognosis forall peri-ampullary cancers.

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Prognostic Factors

For Pancreatic CA

Patients with diploid tumors fared significantly better thananeuploid tumors

 Tumors with p53 mutations have worse prognosis  Tumors with DNA mismatch repair mutations, so-called

RER+ tumors, are associated with improved prognosis

Post-op adjuvant chemotx and radiation tx has been shown

to improve survival 5-FU and external beam radiation tx appear to be indicated

after pancreaticoduodenectomy for pancreatic adenoCA

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Prognostic Factors

Bile duct CA or cholangiocarcinoma Medial survival was 22mos for distal bile duct tumors, with a

5-yr survival of 28% Surgical resection, negative microscopic margins, pre-op

nutritional status, and absence of post-op sepsis were the bestpredictors of improve outcome  Adjuvant chemoradiation does not appear to prolong survival

 AdenoCA of the ampulla of Vater 2nd most common peri-ampullary malignancy, has a higher

resectability rate and a better prognosis  Actuarial 5-yr survival rate at 38% Favorable factors: no peri-op BT, negative lymph node

status, and well-differentiated or moderately differentiatedtumors

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Prognostic Factors

 AdenoCA of the duodenum

Least common of the peri-ampullary neoplasm

 Associated with the best prognosis

5-yr survival rate of 69%

Negative resection margins and tumors located inthe first and second portion of the duodenum

appear to influence survival favorably 

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 Thank You!