Pancreatic tumors

1. PANCREATIC TUMORS
BY
Dr.NILESH SINHA
{Associate Professor}
DEPT. OF GENERAL SURGERY

2. BENIGN EXOCRINE
PANCREATIC NEOPLASMS

3. WHO CLASSIFICATION
• Serous Cystic Neoplasm
• Serous cystadenoma
• Serous microcystic adenoma
• Serous oligocystic adenoma
• Serous cystadenocarcinoma
• Mucinous Cystic Neoplasm
• Mucinous cystadenoma
• Mucinous cystic neoplasm with moderate dysplasia
• Mucinous cystadenocarcinoma(invasive/non-invasive)
• Intraductal Papillary Mucinous Neoplasm
• Intraductal papillary mucinous adenoma
• Intraductal papillary mucinous neoplasm with moderate dysplasia
• Intraductal papillary mucinous carcinoma (invasive/non-invasive)
• Solid pseudopapillary neoplasm

4. FOUR MORPHOLOGICAL TYPES

5. SEROUS CYSTIC NEOPLASM
• 30% of cystic neoplasm
• Women, 50 - 70 years old
• Low risk of malignancy
• Presentation
• Incidental
• Epigastric pain
• Abdominal fullness
• Weight loss.

6. INVESTIGATIONS
EUS with FNA
o Cytology: Scant cellularity/Bloody
o Biochemistry
 Low CEA.
 Low amylase
 Low CA 19-9
CT
o Polycystic/Microcystic
o Stellate scar or sunburst calcifications
o Internal septate
o Honey comb appearance

7. MANAGEMENT
• Observation for 6-12
months if no symptoms
• Consider resection if:
o> 4 cm and symptomatic
oNo definite diagnosis.
oRapid growth.

8. MUCINOUS CYSTIC NEOPLASMS
• Female
• Middle age 30 - 50 years
• There is association with KRAS
mutation
• Body or tail of the pancreas 90%
• Presentation:
• Abdominal discomfort.
• Recurrent Pancreatitis
• Gastric outlet obstruction

9. INVESTIGATIONS
• CT/MRI:
• Thick cyst wall
• Smooth sharp boundaries.
• DO NOT communicate with pancreatic
ductal system.
• Pancreatic duct dilation
• Eggshell calcification
• EUS with FNA:
• Viscous fluid
• CEA
• Adenoma > 200 ng/mL
• Mucinous cystadenocarcinoma >
6000ng/mL

10. MANAGEMENT
• Observation for small tumors.
• Surgical resection if any of the
following:
• >3 cm
• Main duct dilation
• Mural nodule.
• Follow up
• Non invasive: Annually the first years.
• Invasive:
• Every 4 month the first 2 years.
• Biannually until year 5

11. INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM
• Male = Female 50 - 70 years.
• Head > Body
• Presentation:
• Abdominal pain.
• Pancreatitis.
• Weight loss.
• Jaundice.
• New onset diabetes.

12. • Types:
• According to the affected duct
• Main duct type.
• Branch duct type.
• According to the dysplasia
• Adenoma
• Borderline
• Carcinoma in situ.
• Frankly invasive.
• CT
• Main pancreatic or duct dilation.
• Involvement of any part of the
pancreas or the whole pancreas
• Continuity of cyst with ductal
system
• Irregular and poorly demarcated

13. MALIGNANT EXOCRINE
PANCREATIC NEOPLASMS

14. CLINICAL PRESENTATION

15. PHYSICAL EXAMINATION
• Icterus
• Palpable distended gall bladder (1/3rd)
• Courvoisier’s Law – Obstructive jaundice with palpable gall bladder is
seldom due to gall stones
• Exceptions – double stone impaction, primary cbd stones, oriental
chalangiohepatitis
• Widespread disease
• Virchow’s /left supraclavicular nodes
• Sister Mary Joseph /Periumbilical nodes
• Blumer shelf/ perirectal nodes
• Trosseau syndrome/ Migratory thrombophlebitis

16. INVESTIGATIONS
• Hepatic function evaluation
• Coagulation profile
• Nutritional assessment (Albumin)
• Tumour markers
• CEA
• CA 19-9

17. IMAGING MODALITIES
1. Ultrasound - first imaging
investigation in a patient
presenting with obstructive
jaundice
2. CT - PANCREATIC PROTOCOL
• Neutral oral contrast like water
is used. Positive contrast may
compromise the three
dimensional image
• Thinnest possible sections
<3mm preferably 0.5-1mm

18. • CT tells us the Tumour location, size,
vessel involvement, local resectability,
Nodal involvement, Metastatic status
• Scan acquisition time –
• Pancreatic parenchymal phase at
40-50s
• 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
sign
• PET CT – IOC for staging

19. SURGERY
• 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%

21. CHEMOTHERAPY
• More than 80% of patients with pancreatic cancer present with locally
advanced or metastatic disease and are primarily managed with
chemotherapy.
• Gemcitabine has been the standard of care for the treatment of
metastatic pancreatic.
• 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.

22. PALLIATIVE THERAPY
• Biliary obstruction
ERCP with metal stent placement
Roux-en-Y hepaticojejunostomy.
• Delayed gastric emptying
Endoscopic stenting
Preventive gastrojejunostomy
• Pain relief
Antiinflammatories/ long-acting opioids
Celiac nerve block
CT-guided percutaneous neurolysis

23. ENDOCRINE
PANCREATIC NEOPLASMS

24. INTRODUCTION
• 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
decarboxylation cell.
Alpha cells (A)
Beta cells (B)
Delta cells (D)
F or polypeptide cells (PP)

25. CELL TYPES AND DISTRIBUTION
CELL TYPE HORMONE PRODUCE ENDOCRINE TUMOUR/
SYNDROME
DISTRUBUTION
THROUGHOUT THE
PANCREAS
ALPHA (A) GLUCACON GLUCAGONOMA UNIFORM THROUGHOUT
THE BODY/TAIL
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

26. RISK FACTORS
• SYNDROMIC
• MEN 1 (Wermer syndrome)- parathyroid hyperplasia, pituitary tumours, pancreatic
endocrine tumours (30-80 % of patient with MEN 1)
• Von Hippel-Lindau
• Neurofibromatosis
• Tuberous sclerosis complex (TSC)
• GENETICS
• 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

27. INSULINOMA
• 60% of all pancreatic endocrine
tumours
• Average age at diagnosis 45 years
• Men and women are equally
affected
• Equally distributed in the head,
body, and tail of the pancreas
• 90% < 2 cm in size
• Typically hypervascular
• Malignancy occurs in 10% of the
cases
• 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
hypoglycemia

28. • CATECHOLAMINE RELEASE causes
trembling, sweating, palpitations,
nervousness, hunger, weight gain
• NEUROGLYCOPENIC SYMPTOMS like
headache lethargy dizziness diplopia
amnesia
• 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
secretion)
• DIAGNOSIS
• CT and MRI for larger tumors
• EUS can detect small tumors (<2 cm in
size)
• Angiography showing a “blush”
CLINICAL FEATURES INVESTIGATIONS

29. GASTRINOMA
• 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
is gastrinoma)
• 90% of gastrinomas are located within
PASSARO'S TRIANGLE
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

30. LABORATORY FINDINGS
• fasting serum gastrin level 200-1000
pg/mL
• H2 blockers should be stopped 1
week prior to testing, and PPI 3
weeks
• basal acid output > 15 mEq/L
ENDOSCOPY
• multiple ulcers
• large gastric rugal folds
• mucosal edema
• jejunal hypermotility
- Severe form of peptic ulcer disease
• refractory to standard
treatment
• atypical location – jejunal ulcers
- upper abdominal pain
- GI bleeding
- weight loss, nausea, vomiting
- GERD
- Diarrhea relieved by NG suction
CLINICAL FEATURES

31. VIPOMA
• 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

32. 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
• Dehydration
• Electrolyte abnormalities
• Metabolic acidosis (due loss of large amount of
bicarbonate from pancreatic secretion )
WDHA
• Watery
• Diarrhea
• Hypokalemia
• Achlorhydria
LABORATORY FINDINGS
Serum levels of VIP > 150 pg/mL
after an overnight fast
LOCALIZATION
CT or SRS
Intraoperative U/S will localize most
tumors

33. GLUCAGONOMA
• 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
malnutrition.

34. CLINICAL PRESENTATION
• Weight loss
• Hyperglycemia, with 76-94% having
diabetes
• Normochromic normocytic anemia
• Fat-soluble vitamin deficiency
• Hypoaminoacidemia
• Thromboembolism
• Diarrhea
• Vulvovaginitis
LABORATORY FINDINGS
Fasting glucagon level > 50
pmol/L
LOCALIZATION
CT easily detects them
Angiography is also successful
because of vascularity

35. ROLE OF TUMOUR MARKERS
• A variety of tumor markers have been proposed for functional and
non-functional pNETs.
• 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.

36. 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.
TARGETTED THERAPY
• Everolimus an oral mTOR signaling inhibitor, has shown some effect in treating
pNETs.
• Sunitinib is an oral tyrosine kinase inhibitor that is known to target VEGF
receptors.

37. THANK YOU