1. Dr. Amit Goswami

2.  Incidence : 1-10/million
 Age : 30-60years
 Origin:
-Immature pancreatic stem cell
-APUDomas
 Functional /non functional
 Insulinoma and gatrinoma 70%-90%
 Hereditary syndromes

5.  Most common 60%
 Origin- β cells
 F>M
 90% benign, 10% malignant
 Most solitary, 10% multiple
 21% MEN 1 – insulinomas
 The median age at diagnosis- 47yrs

6.  Whipple’s triad
 Worse in morning
 Weight gain
 Diagnosis:
-72hrs fasting test
 Neuroglycopenic symptoms
 Serum glucose<45mg/dl
 Serum level of insulin >5μU/L
 Serum C-peptide(>0.7ng.ml),Proinsulin

7.  Non invasive
 Helical / multi slice CT scan - 82-94% sensitivity.
 MRI with gadolinium - 85%
 DOTATOC scan
 SRS -46%

8.  Selective angiography
 EUS – 77%, EUS guided FNA
 Portal Venous sampling-80%
 Calcium angiogram-90%
 IOUS-90%

9.  Surgical treatment
◦ Therapy of choice
◦ Localisation+Removal with minimal morbidity
◦ Preoperative management
 Optimization of hypoglycemia
 Diazoxide

10.  Enucleation-
• <2cm any part of pancreas, not intimately associated
with the main pancreatic duct
• 2-5 cm if located in the pancreatic head
 Spleen-preserving distal pancreatectomy - tail
of the pancreas
 Whipple procedure- malignant tumours in the
head

11. Gastrinoma

12.  Second most frequent
 1-2 / million
 60% malignant
 75 % sporadic
 M>F
 Average age -50 years,5 to 10 years earlier in MEN-1
 25 % MEN-1
Oberg K, Eriksson B. Endocrine tumours of the pancreas. Best Pract Res Clin Gastroenterol
2005;19:753–81.

14.  Clinical Features
– Abdominal Pain 70%
– Diarrhea 70%
– Heartburn 50%
– Nausea 25%
– Vomiting 20%
– Weight Loss 15%

16.  Fasting Serum Gastrin (Cessation of PPI)
 Basic acid output
 Provocative test(Secretin stimulation test)
-Rise by 200 pg/mL or more

17. ◦ SRS: Imaging modality of choice
◦ 67Ga-DOTATOC
◦ MRI
◦ CT scan
◦ US
◦ Endoscopic US with FNA
◦ Angiography
◦ IOUS

18.  Medical management
 Surgical management
 Localization and removal
 Whipple’s
Distal pancreatectomy
Lymphadenectomy
Bartsch et al., 2005
10-year survival rate
Sporadic ZES 95%
ZES/MEN 1 86%.

19.  Cytoreductive surgery/Radiofrequency ablation
 Hepatic artery embolization/Chemoembolizaion
 Chemotherapy(Streptozotocin and doxorubicin +/-
5-fluorouracil): Rapidly growing tumors
 Somatostatin analogue/Interferon: Slow growing

20. VIPOMA(Verner-Morrison
Syndrome )

21.  0.05-0.2 new cases per million adults
 Third most common neuroendocrine tumor of the
pancreas
 Solitary, found in body or tail.
 2/3 malignant
 Male-to-female ratio in children - 1:1,
in adults. - 1:3

22.  Constant features
◦ Watery Diarrhea
◦ Hypovolemia
◦ Hypokalemia
◦ Acidosis
 Variable features
◦ Achlorhydria or hypochlorhydria
◦ Hypercalcemia
◦ Hyperglycemia
◦ Flushing with rash.

23.  Diagnostic triad
◦ Secretory diarrhea
◦ High levels of circulating VIP > 150pg/ml
◦ A pancreatic tumor
 Localization
◦ SRS - 91% of primary tumors and 75% of metastases.
Nikou GC, et al. Hepatogastroenterology 2005
◦ Endoscopic ultrasound.
◦ CT
◦ MRI
◦ Arteriography
◦ IOUS

24.  Management
 Correction of metabolic abnormality
Octreotide
Distal pancreatectomy/Debulking

25. Glucagonoma

26.  Tumor of islet alpha cells
 Mainly body and tail
 1% of all neuroendocrine tumors
 Mean age of 55 years (19-84 years).
 M:F - 1:3
 Nearly all are malignant

28.  Plasma glucagon >1000pg/ml: Diagnostic
 Enhanced CT scan
 MRI
 SRS
 70%- Metastasis at presentation

29.  Pre-op preparation
 Distal pancreatectomy
 HACE
 Chemotherapy

30. somatostatinoma

31.  Rare
 70% to 90% of tumors – malignant
 Location – usually head
 Clinical findings – unpredictable
◦ Diarrhea
◦ Gallstones
◦ Steatorrhea
◦ Mild diabetes

32.  Localization
◦ CT
◦ MRI
◦ Arteriography
◦ SRS

33.  Management
-Whipple’s
-Debulking
-Cholecystectomy

34.  Incidence :15%-50%
 Malignant ,Metastasis-60%
 Complaints
◦ Abdominal pain
◦ Jaundice
 Surgical resection
 Prognosis - poor
 Resistant to chemotherapy
 Hepatic embolisation.

36.  Pancreatic endocrine tumors -80% MEN 1
 Patients with MEN 1 -decreased life expectancy
 Nonfunctioning pancreatic endocrine tumors –most
common 80-100% of cases
 Gastrinomas -most common functional tumor
- 60% of MEN 1

37.  Surgical Treatment Options
◦ Role of surgery in MEN 1 : controversial
◦ Surgery may be indicated in patients with no distant
metastases
 The goals of surgical therapy
◦ Controlling the symptoms of hormone excess
◦ Safely resecting the maximal amount of tumor mass
possible
◦ Preserving the maximal amount of pancreatic parenchyma
possible

39.  Activation of mTOR and VEGF pathways
 Everolimus+/-Octreotide:Moderately effective(PhaseI/
II trial)
 Role of Sumitinib(Phase III trial)
 Combination of TACE and resection more effacicous
than each procedure alone
ASCO Gastro-intestinal cancer sympossium,2010

40. Just a Sunrise!
Journey
Continues……..