Pancreatic Neoplasms - Endocrine - Core/Disease

Question 1

What is the workup for PNETs?

Answer 1

• H&P
• Biochemical workup
• Localization imaging

Question 2

What symptoms are associated with a nonfunctional PNET?

Answer 2

• Often asymptomatic
• Possible symptoms include:
  
  • Abdominal pain
  • Jaundice
  • Anorexia
  • Weight loss

Question 3

What symptoms are associated with an insulinoma?

Answer 3

Classic Whipple Triad

• Hypoglycemia
• Neuroglycemia (diaphoresis, confusion)
• Resolution of symptoms with eating

Question 4

What symptoms are associated with a gastrinoma?

Answer 4

• Abdominal pain
• Diarrhea (70%)
• Refractory PUD

Question 5

What symptoms are associated with a glucagonoma?

Answer 5

The 4 Ds

• Diabetes
• Dermatitis
  
  • Necrolytic migratory erythema
• DVT
• Depression

Question 6

What symptoms are associated with a somatostatinoma?

Answer 6

• Diabetes
• Cholelithiasis
• Malabsorption; steatorrhea
• Abdominal pain (39%), Jaundice (28%), GI Bleed (22%)

Question 7

What symptoms are associated with a VIPoma?

Answer 7

Verner-Morrison Syndrome aka WDHA syndrome

• Watery Diarrhea
  
  • >5L/day
• Hypokalemia
• Achlorhydria

Question 8

What is the biochemical workup for a nonfunctional PNET?

Answer 8

• Chromgranin A (>300 ng/mL)

OR

• Pancreatic Polypeptide (>200 pg/mL)

Question 9

What is the biochemical workup for an insulinoma?

Answer 9

• Insulin:glucose during fasting
  
  • >0.3 is diagnostic
• 72 hour fast - Gold Standard
  
  • Glucose < 55
  • Insulin 3+
  • C-peptide > 0.2
  • Proinsulin 5+
  • Sulfonylureas neg

Question 10

What is the biochemical workup for a gastrinoma?

Answer 10

• Fasing gastrin >1,000 is diagnostic
• If <1,000 perform secretin stim test
  
  • Overnight fast
  • IV secretin bolus (2 IU/kg)
  • Gastrin lab obtained at 0, 2, 5, 10, 15 minutes after injection
  • Gastrin increase >200 above baseline is diagnostic

Note: Gastrin levels are affected by PPI. Stop PPI prior to testing.

Question 11

What is the biochemical workup for a glucagonoma?

Answer 11

Fasting glucagon level

• > 1,000 is diagnostic
  
  • Normal fasting level is <100

Question 12

What is the biochemical workup for a somatostatinoma?

Answer 12

Fasting somatostatin level

• >160 is diagnostic

Question 13

What is the biochemical workup for a VIPoma?

Answer 13

Serum VIP level

• 225-2,000 is diagnostic

Question 14

How are PNETs localized preoperatively?

Answer 14

1. CT/MRI
2. EUS
   
   • Better for small lesions
   • Can perform FNA at same time
3. Somatostatin receptor imaging (Octreoscan)
   
   • Does not work for insulinomas (no somatostatin receptors)
4. SPECT/CT
5. Angiogram
6. Portal venous sampling
   
   • Ca stimulates insulin release
   • Secretin stimlates gastrin release

Question 15

Preoperative PNET localization was unsuccessful. What can be done in the OR to help localize the lesion?

Answer 15

1. Intraoperative US
2. Tactile sensation
3. Duodenotomy for direct visualization
   
   • Last resort for gatrinomas

Question 16

Nonfunctional PNET Surgical Treatment

Answer 16

SAFE ANSWER: RESECT THEM ALL

• Head: Pancreaticoduodenectomy + regional nodes
• Distal: Distal pancreatectomy + splenectomy + regional nodes

Technically, if <2 cm can consider observation

Question 17

Insulinoma Treatment

Answer 17

Prior to surgery, stabilize glucose levels with:

• Diet and/or
• Diazoxide and/or
• Everolimus

Surgery by location/depth

• Peripheral/exophytic
  
  • Consider enucleation
• Deeper/invasive
  
  • Head: Pancreaticoduodenectomy
  • Distal: Distal pancreatectomy

Question 18

Gastrinoma Treatment

Answer 18

Resection based on localization

• Duodenum: Duodenotomy, local resection/enucleation, periduodenal LN
• Head:
  
  • Exophytic: enucleation
  • Deeper: Pancreaticoduodenectomy
• Distal: Distal pancreatectomy + splenectomy + regional nodes

Question 19

Somatostatinoma Treatment

Answer 19

Resection by location

• Head: Pancreaticoduodenectomy + regional nodes
• Distal: Distal pancreatectomy + splenectomy + regional nodes

Question 20

Glucagonoma Treatment

Answer 20

Before Surgery

• Octreotide preoperatively
• Symptom management

Resection based on location

• Head: Pancreaticoduodenectomy + regional nodes
• Distal: Distal pancreatectomy + splenectomy + regional nodes

Question 21

VIPoma Treatment

Answer 21

Before Surgery

• Octreotide preoperatively
• Correct dehydration and electrolyte imbalances

Resection based on location

• Head: Pancreaticoduodenectomy + regional nodes
• Distal: Distal pancreatectomy + splenectomy + regional nodes

Question 22

What is treatment plan in metastatic PNET?

Answer 22

IF complete resection possible

• Resect both the primary and the metastasis

IF unresectable but asymptomatic

• Observe (intermittent biochemical markers and imaging)
• Consider octreotide

IF unresectable and symptomatic

• Symptom management
• Consider octreotide
• Palliative options

Question 23

What is the follow-up for PNETs?

Answer 23

Standard post-op visit

THEN

• Annually
  
  • H&P
  • +/- biochemical makers as indicated
  • +/- imaging as indicated