Tumours of Pancreas😍

Neuroendocrine Tumours of Pancreas

• Chromogranin

• Synaptophysin

• NSE

INSULINOMA:

• M/c pancreatic endocrine tumour.

• 90% benign.

• Tumour of β-cells.
• Equally distributed in pancreas.

Investigations:

• Fasting insulin increased.

• C-peptide increased.

• 72-hour fasting test (Gold standard).

• Endoscopic USG (EUS):
• Best to localize.

Treatment: 

• Enucleation.

Whipple’s Triad:

• Fasting hypoglycemia symptoms.

• Blood sugar <40 mg/dL.

• Rapid resolution on giving glucose.

GLUCAGONOMA:

4 Ds of Glucogonoma

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Drugs causing DM:

• Steroids

• Thiazide (also gout) > Loop diuretics

• Niacin (also gout)

• Phenytoin

• IFN alpha

• Protease Inhibitors

• Clozapine → atypical

• β Agonists (opp. to β blockers)

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GASTRINOMA:

• Tumour of G cells (Gastrin producing) leading to Zollinger-Ellison Syndrome.

• 80% – Sporadic

• Age: 30–50 years

• Females > Males

• M/c pancreatic tumour in MEN 1 Syndrome.

• 70% malignant.

• Most common metastasis site → Liver

Passaro’s Triangle (Gastrinoma Triangle):

• Boundaries:
• Common Hepatic Duct (CHD) & Cystic duct junction.
• D2-D3 junction.
• Junction of head & neck with body of pancreas.

• Contents:
• 1st part of duodenum (D1)
• Head of pancreas
• Lymph nodes

• Significance:
• M/c site for gastrinoma: Wall of D1.
• Gastrinomas outside Passaro’s triangle are more aggressive.

Clinical Features:

• Hypergastrinemia:
• Stimulates parietal cells → ↑ Acid

• Abdominal pain

• Peptic ulcer = Most common manifestation
• Recurrent ulcers.
• Ulcers at atypical locations.

• Diarrhea.

• Malabsorption.

Indicators of Gastrinoma

• Unusual location of peptic ulcer

• Refractory peptic ulcer (not responding to medical therapy)

• Recurrence

• Diarrhea – Osmotic or secretory

• Multiple duodenal ulcers (less aggressive)

Associated Tumors

• Gastric carcinoids more common in MEN 1

• Most common carcinoid in MEN 1 → Duodenal carcinoid
• Seen in 30% of patients
• Defective menin gene on Chromosome 11
• Better prognosis

To confirm diagnosis of Zollinger-Ellison Syndrome (ZES)

• Gastric pH < 2 +

• Serum gastrin > 1000 pg/ml
• Normal < 100 pg/ml

Investigations:

• Gastrin radioimmunoassay

• Stop acid-suppressants for 48 hrs before testing

• Serum gastrin >1000 pg/mL (Diagnostic).

• If serum gastrin <1000 pg/mL:
• IV Secretin/pentagastrin stimulation test:
• Increase by >200 pg/mL suggests Gastrinoma.
• (Normally: Secretin → ↓ Gastrin
• In gastrinoma: Secretin → ↑ Gastrin)

For localization

• Initial investigation: CT or MRI

• Best investigation: Endoscopic ultrasound
• Useful in diagnosing duodenal gastrinoma in MEN 1
• Most common metastasis → Liver

• If tumor is not localized:
• Somatostatin receptor scintigraphy
• (Octreotide scan)
• Confirmatory imaging

Management: 

• Surgery, Chemotherapy (if malignant).

Medical

• MEN 1 Gastrinoma:
• High dose PPI (40–80 mg)

• Other gastrinomas → Surgical management

Surgery

Tumor Resection:

• Pancreas head → Enucleation

• Pancreas body/tail → Distal pancreatectomy

• Duodenum → Full thickness excision

• Gastrectomy → Not required

Prognosis

Bad Prognosis Indicators:

• Shorter disease duration

• High gastrin levels

• Large pancreatic tumors

• Metastasis

Good Prognosis:

• Gastrinoma in MEN 1

Pancreatic Ductal Adenocarcinoma

• M/c exocrine tumour of pancreas.

Risk Factors:

• Smoking, Obesity, DM, African American, Alcohol.

• Hereditary pancreatitis (PRSS gene).

• Tropical calcific pancreatitis (SPINK I gene).

• Chronic pancreatitis.

• Syndromes: Peutz-Jeghers syndrome (>100 times risk).

Genetic Mutations (in order of occurrence):

1. KRAS (1st & M/c)

2. CDKN2A

3. SMAD4

4. P53 (Last)

5. Mnemonic: K53

Clinical Features:

• Often presents as periampullary cancers.

• Common presentation:
• Obstructive jaundice with palpable GB
  (Courvoisier’s law).

Types (Location):

1. Head of pancreas (M/c).

2. Ampullary variety:
1. Waxing & waning of jaundice + Melena.

3. Distal CBD cholangiocarcinoma.

4. Duodenal adenocarcinoma.

Investigations:

• CECT: IOC.

• Duodenography: Frostberg reverse 3 sign.

• PET-CT: IOC for staging.

• Ca 19-9: Tumour marker.

• Transgastric ultrasound guided fine-needle aspiration cytology (FNAC)
• Biopsy

• MRCP: Double duct sign

• First image
• Dilated CBD + Pancreatic duct
• Open at ampulla of Vater
• Seen in Periampullary carcinoma / Pancreatic head carcinoma

• Second image
• Pancreatic head mass
• pulls duodenal wall
• loss of C curve
• C loop widening
• reverse 3 sign

Treatment:

• Resectable tumour (Head of pancreas): 
• Whipple’s surgery (Pancreaticoduodenectomy).

• Chemotherapy:
• Gemcitabine + Capecitabine.
• mFOLFIRINOX (better results).

Whipple’s Surgery (Pancreaticoduodenectomy):

• Incision: Rooftop/Chevron incision.

• Pylorus preserving Whipple’s: 
• Decreased chances of dumping syndrome.

• Structures removed: 
• Distal CBD, GB, Head of pancreas, Duodenum, Part of jejunum.
• Distal stomach (antrum + pylorus) → if pylorus-preserving, stomach is kept

• 3 Anastomoses:



• PHD → Order
• Pancreaticojejunostomy (PJ) → max mortality
• Choledochojejunostomy (HJ)
• Gastrojejunostomy (DJ)

• Complications:
• Altered gastric emptying (M/c).
• Hemorrhage.
• Pancreatic fistula.
• Wound infection.
• Anastomotic leak
• M/c cause of death
• M/c site: PJ

Pancreatic Cystic Neoplasm

Intraductal Papillary Mucinous Neoplasm (IPMN):

Ohashi’s Triad

Diagnosis: 

• ERCP.