The document discusses pancreatic neuroendocrine tumors. It covers types like insulinomas, gastrinomas, vipomas, glucagonomas, and somatostatinomas. For each type it discusses incidence, location, clinical features, diagnostic tests, management options like surgery or medication, and prognosis. It also covers non-functional pancreatic neuroendocrine tumors and tumors associated with MEN1 syndrome. Surgical resection is the primary treatment when possible but some types are often metastatic at diagnosis.

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……..