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Number of people living in United States with the diagnosis
Neuro-endocrine tumors – 103,312
Gastric – 65,836
Pancreas – 32, 353
Esophagus – 28,664
Hepato-biliary – 21, 427
The best way to assure a negative operation is to operate on a tumorthat doesn doesn’t exist. It is essential for the surgeon to understand keys for diagnosis.
Incidence:
• 1 in 100,000 (clinical)
• 1.5% (autopsy)
Categorize:
• Functional
• Non-functional
• Familial (MEN-1, vHL)
Demography
• No gender bias
• Peak age 30 to 60
Functional (60%)
• Insulinoma
• Gastrinoma
• Glucagonomas
• Somatostainomas
• VIPomas
• Others
Non-functional(40%)
• Secrete pancreatic polypeptide
MEN-1
• 75% gastrinomas, 25% insulinomas
• 40-70% of MEN MEN-1 pts develop NET
• Management controversial
vHL
• Multiple non-functional tumors
• Prevalence > 90%
Surgical resection is mainstay of therapy
Types of resections:
• Enucleation
• Regional - Whipple, Distal panreactectomy
Challenges
• Functional tumors– identifying the lesion
• Non-functional - resectability
Symptoms
• Neuroglycopenic
Diagnosis
• Glucose, insulin, pro-insulin, C C-peptide
Location
• 100% in pancreas
Natural history
• Vast majority are benign
 • Diagnosis made on biochemical testing.
• Pre-op use MRI, EUS; Intra Intra-op ultrasound.
• Expose entire surface
• Intra-op ultrasound is op ultrasound is necessary to guide enucleation for non-palpable tumor
• Tumors are hypoechoic
• IOUS allows assessment of relationship of tumor to pancreactic ducts and vessels
• Lesions in the head can be enucleated if not in direct proximity to the main pancreatic duct
• NET are reddish- - brown against yellow pancrea parenchyma Enucleate on capsule
• Clip of tie divided pancreactic tissue to decrease chance of leak/fistula
 
Symptoms
• Ulcers, heartburn, diarrheaUlcers, heartburn, diarrhea
Diagnosis
• Gastrin, BAO/MAO (acid production), secretin stimulation
Location
• 80% in duodenum; 20% in pancreas
Natural history
• Majority are malignant
• 80% of primary gastrinomas are in the duodenom
• Surgical therapy is full thickness wall resection and peri resection portal lymph node dissection
• 4 cm mid body gastrinoma with main pancreatic duct distorted by the tumor
• Distal pancreatectomy plus splenectomy
NIH study of patients with primary gastrinoma
160 underwent resective surgery
35 patients refused surgery or were not felt to be surgical candidates
Symptoms
• None
Diagnosis
• Appearance on ultrasound, MRI.
• Pancreatic polypeptide, chromogranin
Location
• 100% in pancreas
Natural history
• Majority have malignant potential
72 y/o getting serial scans to follow adrenal incidentaloma. MRI shows
1.8 cm pancreatic body tumor
Patient refuses surgery wants interval scans – stable for 18 months
2 year follow scan shows growth to 2.7 cm. Distal pancreatectomy with
malignant neuroendocrine tumor and 2/13 lymph nodes with metastases
MD Anderson series of 163 patients
Median survival
• Localized disease resectable – 7.1 years
• Localized disease unresectable – 5.2 years
• Metastatic disease – 2.2 years
The liver is the most common site of metastases and often is the only site
Treatment options
• Surgical resection
• Ablative techniques
• Chemoembolization (TACE)
• Systemic therapy
Pancreatic NETs are rare but increasingly diagnosed at small size as incidentalomas on MRI and EUS
CgA and PP best circulating markers
111 Indium-octreoscan best imaging study
Octreotide for symptom control
Best treatment is “More surgery, more surgery, more surgery surgery”
Diffuse disease: foregut, midgut, hindgut
Problem not inability to identify tumor but whether various sites are resectable
Liver primary site of surgical directed therapy
Gastro-intestinal 55%
• Small intestine 45%
• Rectum 20%
• Appendix 17%
• Colon 11 %
• Stomach 7%
Broncho-pulmonary 30%
50 y/o man with typical carcinoid symptoms.
X-ray studies show:
• Mass in distal small bowel
• Bulky mesenteric lymph nodes
• Two large right hepatic metastases extending into left medial segment of
the liver
Decision to operate:
• Remove all disease
• Control symptoms
YES – all carcinoid tumor can be removed successfully
Resection are based on vascular anatomy
Resections may be lobar, segmental, or wedge resections
• Ligate vessels into lobe of liver to be removed
• Divide liver parenchyma
• New tools of vascular staplers, tissue link coagulators, and argon beam coagulators
• 56 y/o man with terminal ileum primary tumor and too numerous to
count liver metastases
• Requested radiofrequency ablation of liver tumors
Decision to operate
• Prevent problems with primary tumor of the bowel
• Unable to impact on disease in liver
Procedure
• Small bowel resection via limited incision
Often > 50% of the liver can be replaced by metastatic cancer
with normal function
No benefit from wedge resection or RFA
Often referred for chemoembolization
Small diamter probes can create relatively large thermal lesions
Maximal size still 4-5 cm
Destroys tissue by heating to boiling point
• Identify the lesion with U/S Guidance
• Position tip of the probe appropriately in the lesion
Follow appropriate algorithm for device in use based on impedance or
temperature
• Plan number of treaments and orientation
• Starts at the most deep central area
• Check the position in three dimensions
• Effective for limited number of nodules with size < 4-5cm
• Can be added to resection of live is one or two metastases in lobe not resected
• Preferred approach is percutaneous if feasible
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