The main pancreatic duct of Wirsung, 3 mm in diameter lies near the posterior
surface of the pancreas. It is related to the bile duct which join to form the ampulla
of Vater which opens on the major duodenal papilla, 8 to 10 cm distal to the
The accessory pancreatic duct of Santorini opens into the duodenum at the minor
1. Digestive: Trypsin breaks down proteins to lower peptides. Amylase
hydrolyses starch and glycogen to disaccharides. Lipase breaks
down fat into fatty acids and glycerol.
• Normal serum amylase – 25 to 115 U/L
• Normal serum lipase – 73 to 393 U/L
2. Endocrine: Insulin helps in utilizations of sugar in the cells. Deficiency
of insulin results in hyperglycaemia and is called diabetes mellitus.
3. Pancreatic juice: It provides appropriate alkaline medium (pH 8) for
the activity of the pancreatic enzymes.
12. SEROUS CYSTIC NEOPLASM
• 30% of cystic neoplasm
• Women, 50 - 70 years old
• Low risk of malignancy
• Epigastric pain
• Abdominal fullness
• Weight loss.
EUS with FNA
o Cytology: Scant cellularity/Bloody
Low CA 19-9
o Stellate scar or sunburst calcifications
o Internal septate
o Honey comb appearance
• Observation for 6-12
months if no symptoms
• Consider resection if:
o> 4 cm and symptomatic
oNo definite diagnosis.
15. MUCINOUS CYSTIC NEOPLASMS
• Middle age 30 - 50 years
• There is association with KRAS
• Body or tail of the pancreas 90%
• Abdominal discomfort.
• Recurrent Pancreatitis
• Gastric outlet obstruction
• Thick cyst wall
• Smooth sharp boundaries.
• DO NOT communicate with pancreatic
• Pancreatic duct dilation
• Eggshell calcification
• EUS with FNA:
• Viscous fluid
• Adenoma > 200 ng/mL
• Mucinous cystadenocarcinoma >
• Observation for small tumors.
• Surgical resection if any of the
• >3 cm
• Main duct dilation
• Mural nodule.
• Follow up
• Non invasive: Annually the first years.
• Every 4 month the first 2 years.
• Biannually until year 5
18. INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM
• Male = Female 50 - 70 years.
• Head > Body
• Abdominal pain.
• Weight loss.
• New onset diabetes.
19. • Types:
• According to the affected duct
• Main duct type.
• Branch duct type.
• According to the dysplasia
• Carcinoma in situ.
• Frankly invasive.
• Main pancreatic or duct dilation.
• Involvement of any part of the
pancreas or the whole pancreas
• Continuity of cyst with ductal
• Irregular and poorly demarcated
24. IMAGING MODALITIES
1. Ultrasound - first imaging
investigation in a patient
presenting with obstructive
2. CT - PANCREATIC PROTOCOL
• Neutral oral contrast like water
is used. Positive contrast may
compromise the three
• Thinnest possible sections
<3mm preferably 0.5-1mm
25. • CT tells us the Tumour location, size,
vessel involvement, local resectability,
Nodal involvement, Metastatic status
• Scan acquisition time –
• Pancreatic parenchymal phase at
• Portal venous phase at 65-70s
(relation of tumour to the vessels)
• MRCP/ERCP – Double duct sign
• Xray – Widening of C loop
• Duodenography – Frosberg inverted 3
• PET CT – IOC for staging
• For tumors involving the head of the pancreas, pancreaticoduo-
denectomy is the procedure of choice.
• Walter Kausch was the first to successfully perform
pancreaticoduodenectomy in Berlin 1912.
• Allen Whipple popularized the operation in US in 1935.
• Operative mortality of >25% and morbidity of >50%
31. • Tumors arising in the body and tail of the pancreas are rarely
resectable at the time of presentation. The survival is significantly
shorter because of the more advanced nature of resected tumors.
• Involvement of the celiac axis is a contraindication to resection.
• For resectable tumors, distal pancreatectomy and en bloc
splenectomy should be performed.
• The distal pancreas and spleen are devascularized and the neck of the
pancreas is divided.
• Laparoscopic distal pancreatectomy (LDP) is increasingly used for
benign conditions and its use for the treatment of PDAC remains to
be fully validated although it has shown significantly increased
32. • Delayed gastric emptying is characterized by the need for prolonged nasogastric
decompression or inability to tolerate oral intake.
• An underlying structural abnormality is ruled out with imaging and endoscopy.
• Enteral feeding with a feeding tube placed during surgery is used to maintain
nutrition while waiting for stomach function to return.
33. A pancreatic fistula or a leak is
defined as an output via an
intraoperatively placed drain of any
measurable volume on or after
postoperative day 3, with amylase
>3 times normal serum value.
Most fistulas require no additional
• More than 80% of patients with pancreatic cancer present with locally
advanced or metastatic disease and are primarily managed with
• Gemcitabine has been the standard of care for the treatment of
• A regimen that has shown greater efficacy than gemcitabine is
FOLFIRINOX (5-FU, oxaliplatin, irinotecan, and leucovorin)
• FOLFIRINOX is being used as the neoadjuvant regimen of choice in
patients with borderline resectable pancreatic cancer and has good
performance status who can tolerate this aggressive regimen.
• The pancreatic islets or islets of
Langerhans are the regions of the
pancreas that contain its endocrine cells.
• Islet cells originate from neural crest cells, aka
APUD cells - Amine precursor uptake and
Alpha cells (A)
Beta cells (B)
Delta cells (D)
F or polypeptide cells (PP)
38. CELL TYPES AND DISTRIBUTION
CELL TYPE HORMONE PRODUCE ENDOCRINE TUMOUR/
ALPHA (A) GLUCACON GLUCAGONOMA UNIFORM THROUGHOUT
BETA (B) INSULIN INSULINOMA BODY/TAIL
DELTA (D) SOMATOSTATIN SOMATOSTATINOMA UNIFORM THROUGHOUT
F PP PPOMA UNCINATE PROCESS
D 2 VIP VIPoma/WDHA UNIFORM THROUGHOUT
G GASTRIN GASTRINOMA/ZES NOT PRESENT/SECRETED
IN NORMAL STATE
39. RISK FACTORS
• MEN 1 (Wermer syndrome)- parathyroid hyperplasia, pituitary tumours, pancreatic
endocrine tumours (30-80 % of patient with MEN 1)
• Von Hippel-Lindau
• Tuberous sclerosis complex (TSC)
• Homozygous deletion or silencing of 5′ CpG island methylation; > 90% of gastrinomas
and non-functioning pancreatic tumours.
• LOH (loss of heterozygosity) at chromosome 11q – functional tumours
• LOH at chromosome 6q – nonfunctional tumours
• 60% of all pancreatic endocrine
• Average age at diagnosis 45 years
• Men and women are equally
• Equally distributed in the head,
body, and tail of the pancreas
• 90% < 2 cm in size
• Typically hypervascular
• Malignancy occurs in 10% of the
• Multicentricity occurs in about 10% of cases
and should raise the suspicion of MEN-1
• Release large amounts of proinsulin (C-
peptide and insulin) which cause
41. • CATECHOLAMINE RELEASE causes
trembling, sweating, palpitations,
nervousness, hunger, weight gain
• NEUROGLYCOPENIC SYMPTOMS like
headache lethargy dizziness diplopia
• WHIPPLE'S TRIAD
- low glucose level (<50 mg/dL)
- symptoms of hypoglycemia
- symptoms resolve with
administration of glucose
• LABORATORY STUDIES
• low glucose l levels (< 50 mg/dL)
• insulin levels > 7 U/mL
• C-peptide to confirm endogenous
source of insulin (marker of insulin
• CT and MRI for larger tumors
• EUS can detect small tumors (<2 cm in
• Angiography showing a “blush”
CLINICAL FEATURES INVESTIGATIONS
• 2nd most common
• Mean age of patients is 50 years
• Slight male predominance (60%)
• Gastrinomas produce ZES (Zollinger Ellison
syndrome) by overproduction of gastrin
• Over 60% are malignant (Most common
malignant endocrine pancreatic tumour
• 90% of gastrinomas are located within
1. Junction between the head and neck
of the pancreas
2. Junction of cystic duct with CBD
3. Junction between the 2nd and 3rd
parts of the duodenum
43. LABORATORY FINDINGS
• fasting serum gastrin level 200-1000
• H2 blockers should be stopped 1
week prior to testing, and PPI 3
• basal acid output > 15 mEq/L
• multiple ulcers
• large gastric rugal folds
• mucosal edema
• jejunal hypermotility
- Severe form of peptic ulcer disease
• refractory to standard
• atypical location – jejunal ulcers
- upper abdominal pain
- GI bleeding
- weight loss, nausea, vomiting
- Diarrhea relieved by NG suction
• VIPomas originate from neoplastic D2 cells aka WDHA or
Verner- Morrison syndrome
• Exceedingly rare tumors
• Bimodal age distribution
• most patients are middle aged
• 10% < 10 years
• Usually solitary located in body or tail
• 2/3rd are malignant
45. CLINICAL FEATURES
Profuse, watery, iso-osmotic secretory diarrhea
• May exceed 3 L/day
• Independent of food intake
• Doesn't resolve with NG suction
• Devoid of blood, fat, or inflammatory cells
• Weight loss
• Crampy abdominal pain
• Electrolyte abnormalities
• Metabolic acidosis (due loss of large amount of
bicarbonate from pancreatic secretion )
Serum levels of VIP > 150 pg/mL
after an overnight fast
CT or SRS
Intraoperative U/S will localize most
• Exceedingly rare tumors
• 2-3 times more common in women
• Averaging 5-10 cm
• Highly vascular
• 65-75% are found in the body or tail
• Malignancy occurs in 50-80%
• 5-17% are associated with MEN 1
• Glucagon is a catabolic hormone, and
most patients present with
47. CLINICAL PRESENTATION
• Weight loss
• Hyperglycemia, with 76-94% having
• Normochromic normocytic anemia
• Fat-soluble vitamin deficiency
Fasting glucagon level > 50
CT easily detects them
Angiography is also successful
because of vascularity
48. MIGRATORY NECROLYTIC DERMATITIS
• Found in 2/3 of patients due to severe amino acid deficiency
• Begins as erythematous patches that spread radially
• Bullae develop then slough with bacterial or fungal superinfection
• Healing begins in center, takes 2-3 weeks, leaving hyperpigmented skin
• Exceedingly rare tumors
• Solitary, large tumors > 2 cm
• Patients are usually in their 5th
or 6th decade of life
• Most are in the head of pancreas
• Majority are malignant
• May be associated with
• Somatostatin inhibits pancreatic
and biliary secretions
51. SRS – SOMATOSTATIN-RECEPTOR SCINTIGRAPHY
• Many pancreatic endocrine tumours
overexpress somatostatin receptor
• Used for metastatic disease
• SRS is useful if tumours are not evident in
• Sensitivity is 80% excluding insulinoma.
• In insulinomas somatostatin receptors are
present only at low levels or absent entirely.
52. ROLE OF TUMOUR MARKERS
• A variety of tumor markers have been proposed for functional and
• The most common of these is chromogranin A (CgA), an acid
soluble protein that is found in secretory granules of
neuroendocrine cells, CgA is sensitive with elevated levels present
in 72-100% of patients.
• Others such as neuron-specific enolase (NSE), pancreatic
polypeptide, pancreastatin, and human chorionic gonadotropin
have been proposed but less sensitive.
53. CYTOREDUCTIVE SURGERY
Most pNETs have a relatively indolent course compared to other pancreatic
neoplasms, and that tumor debulking, while not curative, provides the theoretical
advantages of symptom control in functional tumors.
• Everolimus an oral mTOR signaling inhibitor, has shown some effect in treating
• Sunitinib is an oral tyrosine kinase inhibitor that is known to target VEGF