TUMOURS OF

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PANCREAS
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 BASIC ANATOMY AND PHYSIOLOGY
ANATOMY
Pancreas divided into
Head 30%
Uncinate Process
Neck
Body and Tail 70%

Main Duct:
Duct of Wirsung

Accessory Duct:
Duct of Santorini

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 HISTOLOGY
80-90% Exocrine ie via Pancreatic duct
Basic tissue: Acinar tissue -> Organised as lobules
Interlobular & Intralobular ducts-> ductules -> acini
Produce Pancreatic Juice (Digestive Enzymes + Bicarbonate)

10-20% Endocrine ie No duct -> Directly into blood (Portal Vein)

Basic tissue: Islets of Langerhans Distributed throughout as
Clusters
NB: Numerous in tail of pancreas.
Islet Constituents:
75% - Cells Insulin

20% - Cells Glucagon

04% - Cells Somatostatin

(PP Cells) Pancreatic Polypeptide

G Cells Gastrin

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D2 Cells Vasoactive Intestinal Peptide(VIP)
 In response to food secretes digestive enzymes in an alkaline
bicarbonate rich fluid. Within cells enzymes are in inactive form.
Secretion enhanced by:
Secretin, Cholecystokinin, Vagal Stimulation
Pancreatic Juice (Pancreatic Exocrine Secretions) include
Electrolytes:
Cations: Na+, K+, Ca2+, Mg2+, Zn2+
Anions: HCO3-, Cl- and traces of SO42-, HPO42-

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PANCREATIC
TUMOURS :

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 EPIDEMIOLOGY
Incidence about 3-5 per 100,000 per year in each gender

1.7:1 Male to Female ratio

Very poor prognosis Median survival for unresectable

disease is 6 months (80% of patients have unresectable
disease at presentation); overall 5-year survival <3%)

Most common histology is ductal adenocarcinoma (90% of

tumours)

Anatomic distribution: 75% in the head, 20% in the body,

5% in the tail

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 TUMOURS OF EXOCRINE PANCREAS
SOLID (75%) CYSTIC(25 %)
ADENOCARCINOMA(85%) MUCINOUS CYSTIC NEOPLASM
(45%)
INVASIVE/DUCTAL SEROUS CYSTADENOMA(16%)
ACINAR CELL CARCINOMA(5%) INTRADUCTAL PAPILLARY -
PANCREATOBLASTOMA(1%) - MUCINOUS NEOPLASM(IPMN)
(32%)
OTHERS:ADENOSQUAMOUS SEROUS/MUCINOUS
SIGNET RING CELL - CYSTADENOCARCINOMA(29%)
COLLOID
ANAPLASTIC
UNDIFFERENTIATED
TUMOURS OF ENDOCRINE PANCREAS
GLUCAGONOMA ( CELLS)
INSULINOMAS ( CELLS) 60%
SOMATOSTATINOMA ( CELLS/D CELLS)
GASTRINOMA (G CELLS)
VIPOMA (D2 CELLS)
LYMPHOMAS DP
 CLINICAL PICTURE BASED ON ANATOMY
CARCINOMA HEAD OF PANCREAS/PERIAMPULLARY REGION
Short duration of symptoms(1-3 months maximum)

Obstructive jaundice: persistent ,progressive,painless,pruritic

(if periampullary: can slough off intermittent jaundice)
(NB:Although said painless, pain(mild and vague) is often 1 st symptom in Ca Pancreas)

Clay coloured stools

(NB:if mixed with blood :Silvery stool or aluminium paint stools
5%-growth can ulcerate to duodenum- Malena, jaundice subsides)

Fever with chills and rigors (if cholangitis present d/t Obst Jaundice)

Loss of weight and loss of appetite

Discomfort in abdomen (Pain is relatively rare)

Palpable Gallbladder (Courvoisiers Law)-70-75% cases

Liver can be palpable(d/t back pressure)

(NB:if liver is nodular,sharp border, hard consistency->metastasis)
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Others:Lt supraclavicular LN, Ascitis etc(d/t metastasis)
 CARCINOMA BODY AND TAIL OF PANCREAS
Pain dull constant pain in the epigastrium radiating to the back in the region of L1and L2
(d/t infiltration of retroperitoneal nerve plexuses or d/t pancreatic duct obstruction)

Mass abdomen:
Majority of cases are advanced and felt as mass in upper abd. Mass is on left side
involving left hypochondrium/umbilical/ epigastric region.
Does not move with respiration(because it is retroperitoneal)
Does not fall forward on knee elbow position
Can get above the swelling.
On percussion: Resonant note(d/t stomach anteriorly)

Gross weight loss(in 3-6 mths).Asthenia,Anorexia,Gen. weakness

.
Steatorrhoea, malabsorption : Exocrine insufficiency with duct obstruction

New onset DM

Vomiting : If present d/t duodenal obstruction

Jaundice:Do not occur unless secondaries in liver/LN at porta

Others:Migratory thrombophlebitis Paraneoplastic synd(6%)

(d/t sluggish blood flow l/t thrombus formation),
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Anaemia
Metastatic symptoms: ascites, bone pain, CNS symptoms, dyspnoea
 INVESTIGATIONS FOR PANCREATIC NEOPLASM
CA 19-9
Elevated in 75% of patients with Pancreatic cancer
NB:Also elevated in 10% benign conditions of pancreas, liver, bile duct
Can be used as a marker for tumour recurrence during post-op follow-up
.
USG:First diagnostic study

CECT SCAN:If bile duct dilated and no gall stones, then next CECT
UNRESECTABLE in CT:
Hepatic artery/SMA/Celiac plexus involvment
Enlarged LN outside the boundaries of resection
Ascitis
Distant metastasis
NB:SMV,Portal Vein invasion is not a contraindication

EUS: Used when there is high suspicion for pancreatic cancer , but no
mass is
identified by the CT Scan.
Has added advantage of transluminal biopsy (in cases of advanced tumors for
neoadjuvant chemotherapy)
NB:In normal scenario, tissue diagnosis before pancreaticoduodenectomy is
not required. DP
NB:Problem with preoperative/intraop biopsy is that many pancreatic
cancers are not very cellular and contain a significant amount of fibrous
tissue,so biopsy is misinterpretated as chronic pancreatitis if it dont have
malignant cells.

DIAGNOSTIC LAPROSCOPY:
If all current staging modalities are used , accuracy in predicting resectability is 80%
i.e 1 in 5 is found on table as unresecectable.
Use of diagnostic Laparoscopy with imaging makes accuracy 98%
Avoids 10-30% negative laparotomy in Ca head of pancreas
Avoids as high as 50% negative laparotmies in Ca body and tail of pancreas

MRI PANCREAS WITH MRCP

MRI pancreas is not superior to CT scan; MRCP is useful in delineating biliary system
anatomy especially if the system is not obstructed and there are no therapeutic
indications for ERCP (since there are considerable risks with ERCP)

STAGING
CECT/MRI of the abdomen T, N stage; metastasis to the liver
Endoscopic ultrasound T, N stage
Lungs CXR + CT thorax
Bones bone scan when suspicion is high
Staging laparoscopy for peritoneal metastases, just before definitive operation
for a resectable tumour (since CT/MRI may miss small peritoneal deposits.
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TNM STAGING

STAGE I A
Limited to Pancreas 2cm
STAGE IB
Limited to Pancreas 2cm

STAGE II A
Extend beyond pancreas but does not
Involve arteries,LN
STAGE IIB
Any tumour without artery involved,
But with LN involvment

STAGE III
Unresectable (T4), (i.e artery involved)
But Without distant metastasis.

STAGE IV
Distant Metastasis (Eg Liver,Lung)

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NB:SMA,Coeliac Trunk:Unresectable
 TREATMENT OF PANCREATIC TUMOUR

IMMEDIATE MANAGEMENT
Treat any life-threatening complications such as
cholangitis, pancreatitis, bleeding.

RESECTABLE TUMOURS

BODY/TAIL OF PANCREAS: DISTAL PANCREATECTOMY

HEAD OF PANCREAS: WHIPPLES OPERATION

Pancreaticoduodenectomy for head of pancreas or periampullary
tumour
Removal of the head of the pancreas, duodenum, proximal 15cm of
jejunum, common bile duct, gallbladder, and distal part of the
stomach
Common hepatic duct and pancreas are then anastomosed to the
jejunum, 45-60cm proximal to the gastrojejunostomy.
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DP
 COMPLICATIONS OF WHIPPLES OPERATION
Morbidity rate of up to 20-30% (mostly mild complications)
Mortality rate is 2-7%.

INTRAOPERATIVE/EARLY COMPLICATIONS
Injury to other organs liver, kidney, bowel
Bleeding
Infection / sepsis
Pancreatitis
Pancreatic anastomotic leak (5-20%)
Biliary anastomotic breakdown
Fistulation, pseudocyst formation may occur due to anastomotic
leaks

LATE:
Long-term exocrine insufficiency resulting in malabsorption and
steatorrhoea
Gastric stasis with pylorus-preserving Whipples
Diarrhoea resulting from autonomic nerve injury during lymph node
dissection
Endocrine insufficiency : DM DP
 OTHER CURATIVE SURGERIES

TRAVERSO LONGMIRE PYLORUS PRESERVING

PANCREATICODUODENECTOMY
PANCREATICOJEJUNOSTOMY +
CHOLEDOCHO/HEPATICOJEJUNOSTOMY +
DUODENOJEJUNOSTOMY
NB:Duodenum is cut 2 cm distal to pylorus. And continuity made with
jejunum

FORTNERS REGIONAL PANCREATECTOMY(EXTENDED WHIPPLE)

WHIPPLE + REMOVAL OF SEGMENT OF SUPERIOR
MESSENTERIC VEIN +
RECONSTRUCTION WITH SYNTHETIC GRAFT

TOTAL PANCREATECTOMY
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TREATMENT OF ADVANCED (UNRESECTABLE) PANCREATIC
TUMOUR
PALLIATION :of 3 problems
Pain
Jaundice
Duodenal Obstruction

PAIN: Celiac Plexus block

JAUNDICE:
ENDOSCOPIC APPROACH:( ERCP )
Coated expandible Metallic stent:Last 5 months,but has risk of tumor
ingrowth.
10 F Plastic Stent.Most patients do not require replacement for 3 month.
NB:(Since unresectable Ca patients usually live <1 Year, requirement of
numerous stents is unlikely)
Complications:Recurrent Obstruction, Cholangitis

SURGICAL:
Choledocho Jejunostomy (Prefered approach)
Cholecysto Jejunostomy (ok if cystic duct enters CBD well above the tumour)
Choledocho Duodenostomy (not preferedDP due to proximity of tumour)
TREATMENT OF ADVANCED (UNRESECTABLE) PANCREATIC TUMOUR
DUODENAL OBSTRUCTION:
Occurs late. That too only in 20% patients.
Mx:Gastrojejunostomy (Antecolic)
Loop Gastrojejunostomy +Jejunojejunostomy
Roux-en Y Gastrojejunostomy
(GJ located 50 cm downstream from the hepaticojejunostomy)

NB:In the absence of obstructive picture , routine use of prophylactic GJ when exploration
reveals unresectable tumor is controversial
(GJ has its own complication:delayed gastric emptyng).

SUMMARY
If initial diagnostic laproscopy shows C/I to whipple: Endoscopic stenting + GJ sos
(i.e :When obstruction develops only)
If Laparotomy already performed for assessing resectability and whipple not possible : Surgical
Bypass

OTHER: Steatorrhoea treated by enzymes. Control of DM

PALLIATIVE CHEMOTHERAPY:
Gemcitabine
Improves pain control, Perfomance status , Weight gain.
Survival is improved only by 1-2 months.
NB:Palliative chemotherapy/radiotherapy/chemoradiotherapy
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Not shown to provide good outcomes
BILIARY-ENTERIC BYPASS TO PALLIATE UNRESECTABLE
PANCREATIC CANCER

TRIPLE BYPASS:GASTROJEJUNOSTOMY +
CHOLEOCHO/HEPATICO/CHOLECYSTOJEJUNOSTOMY +
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JEJUNOJEJUNOSTOMY
 ADENOCARCINOMA
Most common neoplasm of the pancreas(70%)
M.C:Infiltrating ductal adenocarcinoma(85%)
Most common between 60 & 80 years of age
Slight male predominance (1.6:1)
RISK FACTORS
Cigarette smoking
High fat diet
Chronic pancreatitis
Diabetes mellitus
Chemical (carcinogen) exposure
Genetic: Hereditary breast and ovarian cancer
BRCA2 families
Hereditary pancreatitis (50X)
Peutz-Jeghers Syndrome,HNPCC
TREATMENT:surgical resection based on site.(WHIPPLE/DISTAL
PANCREATECTOMY)
PROGNOSIS :3 months without treatment
10-20 months with surgery
5 yr survival rate Without Surgery <5%
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After Surgery 20%
 PATHOLOGY

GROSS PATHOLOGY
Most occur in the head of the pancreas (60%)
15% in the body
5% in the tail
20% diffuse

Poorly circumscribed, grey-white or yellowish white, firm mass

Can infiltrate outside the pancreas into other organs

HISTOPATHOLOGY
Arise from ductal (glandular) epithelium

Typically moderately to poorly differentiated adenocarcinoma

Perineural and angiolymphatic invasion common.

Almost always associated with chronic pancreatitis

Histologic variants
Adenosquamous Carcinoma
Anaplastic Carcinoma
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Undifferentiated carcinoma with osteoclast-like giant cells
Adenocarcinoma - Gross

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 ACINAR CELL CARCINOMA

5% of all exocrine tumours.

Associated with :Metastatic fat necrosis (skin rashes)
Eosinophilia
Polyarthralgia

PANCREATOBLASTOMA
Very rare tumour (<1%)
M.C in children (<10 years old)
Good Prognosis.

LYMPHOMA
CLINICAL PICTURE
Similar to adenocarcinoma(vague abdominal pain,weight loss)
TREATMENT
No role for surgical resection.
Endoscopic stenting to relieve jaundice followed by
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chemotherapy is treatment of choice.
 CYSTIC NEOPLASMS OF PANCREAS(25%)
MUCINOUS CYSTIC NEOPLASM(MCN)
M.C in females(90%) . Mostly perimenopausal
M.C.S: Body and Tail( 2/3rd Cases )
M.C Cystic neoplasm(40%)
Considered premalignant:(Can progress to invasive adenocarcinoma)
Form unilocular or multilocular cysts
Never has communication to the Pancreatic ductal system

FLUID ANALYSIS:
High CEA(> 200 ng/ml) suggest malignant transformation
low levels of amylase

CYST FEATURES ON IMAGING:

Thick walled, with no communication with duct,Columnar epithelieum lined
Submucosal layer has cellular stroma similar to ovarian stroma-key pathologic
feature
Nodules or calcifications may be present along cyst wall

TREATMENT:
Surgical Resection (Distal pancreatectomy). Malignancy cannot be rule out with
removal and extensive sampling of tumour
NB:for small lesions, spleen may or may not be preserved.but splenectomy ensures
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removal of the LNs that can potentially be involved.
PROGNOSIS
MCN (especially if <3cm) without atypia :Cured if completely resected.
Moderate dysplasia or carcinoma in situ- Cured by complete resection.
Invasive carcinoma:Prognosis similar to typical ductal
adenocarcinoma.

MUCINOUS CYSTIC NEOPLASM

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 INTRADUCTAL PAPILLARY
MUCINOUS NEOPLASM (IPMN)
Slightly more common in males(Present studies :Equal predominance)
M.C.S :Head involving ampulla of vater/pancreatic duct
Incidence is 25% of cystic neoplasms
Communicates with duct
Ductal epithelium forms a papillary projection into the duct, and
mucin production causes intraluminal cystic dilation of pancreatic
ducts.
Can progress to invasive adenocarcinoma:High malignant potential.(35-40%)
Has high risk for other malignancies:Should ideally undergo Colonoscopy,
close surveillance.

CLINICAL PICTURE
Abdominal Pain or Recurrent Pancreatitis(d/t duct obstruction by thick mucin)
5-10% have steatorrhoea,diabetes,weightloss secondary to pancreatic
insuficiency
Branch duct only involved IPMN may be asymptomatic(& less malignancy
chance)
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 INVESTIGATIONS
EUS, MRCP/ERCP, +/- Intraop Ductoscopy
IMAGING STUDIES:
Demostrates Diffuse dilation of pancreatic duct .
NB:
Can have skip areas-Normal area in btw diseased portions(microscopic
spread)
Pancreatic parenchyma is often atrophic d/t chronic duct obstruction.
May sometimes show only dilated pancreatic duct with no mass.
d/d Chronic pancreatitis:Classic features of calcification,beaded appearance of
duct seen in chronic pancreatitis are not present in IPMN

ERCP will Show mucin egress from bulging papilla (FISH EYE LESION)-
Diagnostic

TREATMENT :
Surgical Resection- Whipples Resection

PROGNOSIS
Borderline tumours, carcinoma in situ tumours after complete resection:Cured
Invasive carcinoma: Better than adenocarcinoma
5 year survival rate :60%
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10 year survival rate is 50%
INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM - IPMN

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INTRADUCTAL PAPILLARY MUCINOUS NEOPLASM
- IPMN

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 SEROUS CYSTIC NEOPLASMS
Common in women
M.C.S :50% head and Uncinate Process, 50% body and tail
Incidence:30% of all cystic pancreatic neoplasms
Almost always benign.(Malignant Potential is <1%)
Can be observed if asymptomatic (no mass effect/ Rapid growth)
Average rate of growth : 0.45 cm/year
50% asymptomatic , detected incidentally
Symptomatic:Mild upper abdominal pain,Epigastric fullness,Moderate
weight loss

Fluid analysis:
Contain thin serous fluid
Does not stain positive for mucin
Low in CEA(<200 ng/ml) ,Amylase

Cyst Features on Imaging:

Well circumscribed cystic mass
Small Septations
Fluid Close to water density
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Central Scar with calcifications(Sometimes only)
 OTHER CYSTIC NEOPLASMS- RARE SCENARIOS
1.SOLID PSEUDOPAPILLARY TUMOR
Typically occur in young women
Cyst are not true epithelial lined cyst , but rather represent a
necrotic/degenerative process.
Histology:Similar to neuroendocrine tumors,but do not stain
positive for neuroendocrine markers-Chromogranin.
Well cicumscribed on CT
Previous names include
Solid and Cystic tumour
Solid and Papillary tumour
Cystic and Papillary tumour
Papillary Cystic tumour

2.DUCTAL ADENOCARCINOMA undergoing cystic degeneartion d/t

central necrosis
3.NEUROENDOCRINE TUMOURS : 5-10%
Contains serosanguinous fluid ,rather than necrotic debris.
Often contains thin serous fluid or DP
cheesy/caseous material if there is
MANAGEMENT OF PANCREATIC CYSTIC NEOPLASMS

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 COMPARISON OF CYSTIC NEOPLASM WITH
PSEUDOCYST
CYSTIC NEOPLASM
History of pancreatitis absent
On imaging may demonstrate a solid component
ERCP will Show mucin egress from bulging papilla in case of IPMN
Mucin content may be present in fluid.
CEA is raised in fluid of mucinous cystic neoplasms
CA 19-9 is high in malignant cystic neoplasms
Percutaneous wall biopsy may show epithelial lining

PSEUDOCYST
History of pancreatitis is often present
Imaging wont demonstrate solid component
Mucin will be absent in fluid and also CEA in fluid.
CA 19-9 will be normal.
Percutaneous wall biopsy will show no epithelial lining.
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 ENDOCRINE TUMOURS
Belongs to group of tumours called APUDOMAS

M.C.S :Body and tail (except Gastrinomas)

20% of pancreatic endocrine tumours are nonfuctional

(ie dont produce hormones or, if producing ,wont cause
symptoms)

Can be associated with MEN syndrome (MEN I)

Parathyroid adenoma
Pitutary Adenoma
Pancreatic tumor
Requires special tests for diagnosis.

Remember:Insulinomas are Mostly Benign(90%)

Gastrinomas are almost always Malignant(50%)
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Glucagonomas are commonly malignant(80%)
 INSULINOMA
M.C Endocrine tumour (60%)
B cell tumor- Hypervascular- mostly <2 cm(1 to 1.5 cm)
M.C Solitary (90%) but can be multicentric(10%)-often as firm nodule.
90% benign, 10% malignant
90% Sporadic, 10% associated with MEN I
NB:Those associated with MEN I are mostly multifocal, Recurrent
Evenly distributed in head , body ,Tail with slight predominance in body,tail

DIAGNOSIS by WHIPPLE TRIAD

1- Symptoms & Signs of hypoglycaemia in fasting state
2- Blood glucose less than 2.8 mmol/l (50mg/dl) during attack
3- Symptoms relieved by glucose

NB:D/D of persistant Hypoglycaemia includes

Insulinoma,
Hepatoma,Hepatocellular damage,
Hypopitutarism,
Addisons Ds,
Large Messenchymal Tumor
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 CLINICAL PICTURE :INSULINOMA
Hypoglycaemia (sweating, dizziness, tremor, hunger)

Weight Gain (Common d/t over eating)

About 85% of patients with insulinoma present with one of the

following symptoms of hypoglycemia:
Diplopia
Blurred vision
Palpitations
Weakness

Hypoglycemia can also result in the following:

Confusion , Amnesia
Abnormal behavior
Unconsciousness

Adrenergic symptoms (from hypoglycemia-related adrenaline release):

Weakness, sweating, tachycardia, palpitations, and hunger
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Seizures
 INVESTIGATIONS:

BIOCHEMICAL STUDIES

Diagnosis of insulinoma is established in 95% of patients during

prolonged fasting (up to 72 h) when the following results are found:

Serum insulin levels of 10 U/mL or more (normal < 6

U/mL)

Glucose levels of less than 40 mg/dL

C-peptide levels exceeding 2.5 ng/mL (normal < 2 ng/mL)

Proinsulin levels greater than 25% (or up to 90%) of

immunoreactive insulin levels

Plasma insulin (u/ml) to Glucose ratio (mg/dl) >0.3 is

diagnostic DP
 IMAGING STUDIES
Real-time transabdominal high-resolution USG: 50% sensitivity

Intraoperative transabdominal high-resolution ultrasonography

with the transducer wrapped in a sterile rubber glove and passed over
the exposed pancreatic surface: Detects more than 90% of insulinomas

Helical or multislice (CT) scanning: 82-94% sensitivity

MRI with gadolinium: 85% Sensitivity

Endoscopic ultrasonography: Detects 77% of insulinomas

Arteriography: Selective arteriography has 82% accuracy, with a 5%

false-positive rate; arteriography with catheterization of small arterial
branches of the celiac system combined with calcium injections and
simultaneous measurements of hepatic vein insulin during each
selective calcium injection localizes tumors in 47% of patients
NB:Insulinomas are hypervascular:So angiogram leads to tumor blush
Calcium inj stimulate insulin release from neoplastic tissue but not from normal islet
cells.
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 MANAGEMENT
PHARMACOLOGIC THERAPY
to prevent hypoglycemia
in patients with malignant tumors, to reduce the tumor burden.

Agents used in this therapy include the following

DIAZOXIDE: Reduces insulin secretion (switch off calcium
channels)
3mg/kg in 3 doses.(600mg)
HYDROCHLOROTHIAZIDE: Counteracts edema and
hyperkalemia secondary to diazoxide and potentiates its
hyperglycemic effect .
OCTREOTIDE: Prevents hypoglycemia (Reduces insulin
secretion)

SURGICAL
Enucleation(since majority are benign)
Distal pancreatectomy/Pancreaticoduodenectomy if tumors are
DP
close to the main pancreatic duct and large(>2cm) tuours
 GASTRINOMAS
2nd M.C endocrine pancreatic tumour.

M.C Endocrine Pancreatic tumour associated with MEN I

NB:25% of Gastrinomas are associated with MEN I

M.C in Males

M.C.S is PASSAROS TRIANGLE

Junction between the head and
neck of the pancreas.
Junction of cystic duct with CBD
Junction between the 2nd and 3rd
parts of the duodenum

50% are multiple

50% are malignant.(Liver(80%)/L.N

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Lungs/Bone)
 CLINICAL PICTURE :GASTRINOMA
Abdominal pain, Severe multiple peptic ulcers in the stomach,
Duodenum and Jejunum.

NB: Ulcers are

Multiple
Resistant/Refractory (Not responding to intensive
medical treatment)
Recurrent(Inspite of treatment)
More prone for bleeding/perforation.
Seen in unusual age

Diarrhoea(20% at time of diagnosis), Steatorrhoea, Hypokalaemia

(d/t acid irritating Small Bowel activity)

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 INVESTIGATIONS :GASTRINOMA
GASTRIN ASSAY >200pg/ml .Often it is>1000 pg/ml
(Normal Level <100-150 pg/ml).
BAO in Gastrinoma is >15 meq/hour(specific of Gastrinoma)
NB: Other causes of Hypergastrinaemia are
Pernicious Anaemia
Treatment with PPI
Renal Failure
G Cell Hyperplasia
Atrophic Gastritis
Incomplete antrectomy
Gastric outlet obstruction
SECRETIN STIMULATION TEST:
Inject 2 Units/kg secretin IV and assess blood samples.
(NB:Done in equivocal cases , when gastrin level is not markedly raised.)

GASTROSCOPY

Rule Out MEN I by checking S.CALCIUM levels prior Surgery.

DP
IMAGING:GASTRINOMA

I.O.C : Somatostatin Receptor Scintigraphy in

combination with CT.

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 TREATMENT :GASTRINOMA
If patients have MEN I :For parathyroid hyperplasia do TOTAL
PARATHYROIDECTOMY and IMPLANTATION OF PARATHYROID TISSUE in
the forearm.

DUODENAL GASTRINOMAS: Growth is often submucosal, But a full thickness

excision of duodenal wall is performed.

SOLITARY PANCREATIC TUMOR WITH NO MAJOR DUCT

INVOLVMENT:Enucleation
If doing pancreatic resection, it is justified only for Solitary Gastrinomas with no
Metastasis (or if liver mets, which can also be removed.)

UNRESECTABLE OR IF TUMOUR CANNOT BE LOCALISED: Highly selective

vagotomy
Chemotherapy:Streptozocin,Doxorubicin,5 FU

POSTOP FOLLOWUP:
Fasting S.Gastrin levels,Secretin Stimulation tests, Octreotide Scan ,CT Scan.

PROGNOSIS
15 year survival rate without liver metastasis:80%
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5year survival rate with liver metastasis: 20-50%
ZOLLINGER ELLISON SYNDROME

TYPE 1:G Cell Hyperplasia with hypergastrinaemia and

peptic ulceration.

Treatment:Partial Gastrectomy with removal of G cell

area.

Type II:Gastrinomas

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 GLUCAGONOMA
Serum Glucagon usually >500 pg/ml
M.C.S :Body and tail, M.C in females
Attains large size of 5-10 cm
17% associated with MEN II
Mostly malignant (80%)

CLINICAL PICTURE:
Diabetes + Dermatitis+ Malnutrition
Diabetes : Usually mild (90%)
Dermatitis: d/t low aminoacid levels
Necrolytic migratory erythema(65%)
NME:manifest as cyclic migrations of lesions with spreading margins and healing
centers typically on the lower abdomen, perineum,perioral area and feet.
Other:Stomatitis,Glossitis,Cheilosis,vulvovaginitis
Malnutrition:Since glucagon is a catabolic hormone.

MANAGEMENT
Preop: Control of diabetes,
Parentral nutrition(aminoacid supplementn)
Octreotide
NB:AA supplementation is imp in conrolling rash.
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Surgery:Resection(Distal Pancreatectomy)
 SOMATOSTATINOMA
M.C.S:Head of pancreas

TRIAD :DM,STEATORRHOEA,CHOLECYSTOLITHIASIS

In a normal subject , ACTIONS OF SOMATOSTATIN include:

In the anterior pituitary gland, the effects of somatostatin are:
Inhibit the release of growth hormone thus opposing the effects of growth hormone-
releasing hormone (GHRH)
Inhibit the release of thyroid-stimulating hormone (TSH)
Somatostatin suppresses the release of gastrointestinal hormones
Gastrin (tumor leads to Hypochlorhydria)
Cholecystokinin(CCK) (tumor leads to steatorrhoea,Gall stones)
Secretin (leads to steatorrhoea)
Motilin
Vasoactive intestinal peptide (VIP)
Gastric inhibitory polypeptide(GIP)
Enteroglucagon
Lowers the rate of gastric emptying, and reduces smooth muscle contractions and blood flow
within the intestine
Suppresses the release of pancreatic hormones
Inhibits the release of insulin( tumor leads to Diabetic Mellitus )
Inhibits the release of glucagon
Suppresses the exocrine secretory action of pancreas.

TREATMENT:Chemotherapy with streptozocin, dacarbazine, doxorubicin or

DP
Watchful waiting' and surgical debulking via Whipple procedure
 VIPoma
Also known as VERNERMORRISON SYNDROME
PANCREATIC CHOLERA
WEAK TEA SYNDROME
WDHA SYNDROME
Arises from D2 Cells
Secretes VIP > 150 pg/ml
M.C.S: Body and tail -Solitary
Usually Malignant
5% associated with MEN I syndrome

C/P: Profound and chronic Watery Diarrhoea

(fasting stool volume > 750 to 1000 mL/day)
Dehydration
Hypokalemia
Achloryhydria
Other C/P :Acidosis,Vasodilation(Flushing and hypotension)
Hypercalcaemia(d/t hyperPTH like actions of VIPoma) and
DP
Hyperglycemia
 TREATMENT :VIPoma

CORRECT DEHYDRATION-IV Fluids/electrolytes/acid base

abnormalities
(KCl, if acidosis severe Soda Bicarb)

SLOW THE DIARRHEA-Octreotide(blocks action of

VIP),Prednisolone

SURGERY TO REMOVE THE TUMOR-Distal Pancreatectomy

METASTATIC DISEASE: peptide receptor radionuclide therapy

(PRRT) can be highly effective. This treatment involves attaching a
radionuclide (Lutetium-177 or Yttrium-90) to a somatostatin analogue
(octreotate or octreotide)
OR
Chemotherapy:Combination of Streptocozin with 5 FU

DP
 THE
END
DP
DP
DP