Neuroendocrine Tumors of the Pancreas
Download
Report
Transcript Neuroendocrine Tumors of the Pancreas
Neuroendocrine Tumors of the
Pancreas
JOSHUA M.V. MAMMEN MD PHD FACS
Overview of Talk
Non-functional Pancreatic Endocrine Tumors
Functional Pancreatic Endocrine Tumors
Nonfunctional Pancreatic
endocrine tumors
Background
58-85% of PET’s are non-functional
Most common location is the pancreatic head
Due to lack of symptoms, tend to present with a large
size
Median survival: 3.2 years (7.1 years if potentially
resectable)
Presentation:
Abdominal pain
Weight loss
Obstructive jaudice
Bowel obstruction
Diagnosis
Usually diagnosed by CT
scan or MRI
Endoscopic ultrasound is
more sensitive for smaller
lesions
Octreotide imaging may
help find additional lesions
75% are secreting
hormones in an occult
fashion (chromogranin A,
pancreatic polypeptitide)
Treatment
Only potentially curative strategy is surgical
resection with regional lymphadenectomy
Imaging studies should be performed to assess for
resectability
Incomplete resection or debulking is not
recommended
Consider chemo or radio or bland embolization of
hepatic metastases in setting of unresectable disease
May consider observation for low grade/slow
growing disease
Treatment
Systemic treatment
Streptozocin based chemotherapy (up to 39% response in
combination)
Octreotide
Functional Pancreatic
Endocrine Tumors
Insulinoma
First described in 1935 by Whipple and Frantz
Most common functional PNET
Triad of symptoms (Whipple’s triad):
Hypoglcemic symptoms when fasting
Blood glucose level of less than 50
Symptom relief with glucose
Insulnoma
10% Malignant
10% Multiple
10% associated with MEN1
Insulinoma: Diagnosis
72 hour monitored fast
Draw plasma glucose, C-peptide, proinsulin, and insulin every
6 hours
Continue test until plasma glucose is less than 45 and patient
has symptoms
Criteria for diagnosis:
Insulin concentration greater than 6 uU/mL
Insulin to glucose ratio greater than 0.3
C-peptide level greater than 0.2 nmol/L
Proinsulin level greater than 5pmol/L
Lack of plasma sulfonylurea
Insulinoma: Diagnosis
Exogenous insulin
Low C-peptide levels
Low proinsulin levels
Oral hypoglycemic agents
Elevated C-peptide levels
Elevated proinsulin levels
Plasma sulfonylurea
Insulinoma: Treatment
Surgical resection is the main treatment
Prior to surgery, control glucose with small meals
and diazoxide
Use intraoperative ultrasound to identify additional
lesions at the time of surgery
Does not require anatomic resection, merely
enucleation
If there is evidence of malignancy, should attempt
formal resection (median disease free survival is 5
years)
Gastrinoma
First described by Zollinger and Ellison in 1955
Secretes gastrin that leads to hyperchlorhydria and
parietal cell hyperplasia
Triad
Atypical peptide ulcerations
Gastric hypersecretion with hyperacidity
Noninsulin producing islet tumor of the pancreas
Gastrinoma
Sporadic gastrinomas (75%) usually present at 45
years old
Majority (60%-90%) are malignant
Location
63% are in the pancreatic head
Presentation
Abdominal pain (75%-100%
Diarrhea (35%-73%)
Heartburn (44%-64%)
Duodenal and Prepyloric ulcers (71%-91%)
Gastrinoma: Diagnosis
Carefully should withdraw PPI use
Fasting gastrin level of greater than 1000 pg/mL and
pH less than 2.5
Diagnosis requires:
Basal acid output greater than 15 mEq/hour
Positive secretin stimulation test
Adminster 2 units/kg intravenous secretin after overnight fast
Draw gastrin levels prior to secretin and at 0, 2, 5, 10, and 20
minutes
Positive is increase in serum gastrin over 200 pg/mL
Gastrinoma: Diagnosis
CT and MRI can localize larger lesions
Endoscopic ultrasound for smaller lesions
Octreotide Imaging
Selective angiography and selective arterial secretin
injection
Diagnosis: Treatment
Initial treatment is PPI or H2 blocker
Perform enuclation or resection with associated
lymph nodes if can be identified
If cannot identify preoperatively, surgical
exploration with intraoperative ultrasound
Isolated liver metastases should be resected
Vasoactive Intestinal Polypeptidoma
Tumors secrete vasoactive intestinal polypeptide
(also known as Vener-Morrison syndrome or watery
diarrhea hypokalemia-achlorhydria syndromeWDHA)
Presentation:
Large volume secretary diarrhea
Electrolyte imbalances (hypocholorhydria)
Flushing
Vasoactive Intestinal Polypeptidoma
60-80% are malignant at presentation
5- year survival is 69%
80% are isolated to the pancreas
Diagnosis:
Fasting plasma vasoactive intestinal polypeptide levels greater
than 500 pg/mL
High volume diarrhea
Imaging studies
Vasoactive Intestinal Polypeptidoma
Treatment is surgical resection with
lymphadenectomy
Need to hydrate and correct electrolytes prior to
surgery
Streptozocin based chemotherapy if unresectable
Glucagonoma
Arise pancreatic alpha
cells
Cause increased
glucagon secretion with
glucose intolerance,
weight loss,
neuropsychiatric
disturbances, venous
thrombosis, and
necrolytic migratory
erythema
Glucagonoma
Diagnosis
Inappropriately elevated fasting glucagon grweater than 500
pg/mL
Biopsy of necrolytic migratory erythema
Imaging to identify location
Somatostatinoma
Arise from delta cells of the pancreas
Majority are sporadic (90%)
Most likely to be located in the pancreas
Presentation:
Diabetes mellitus
Cholelithiasis
Steatorrhea
Weight loss
Anemia
Diarrhea
Somatostatinoma
Somatostatin level greater than 100 pg/mL
Localize with imaging
Typically present late
Treatment is surgical resection with
lymphadenectomy
Tumor debulking for palliation
Always perform cholecystectomy due to cholestasis
Summary
Generally, treatment is surgical resection for
sporadic cases
Functional tumors have specific tests that help in
diagnosis