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islet cell tumor; pancreatic neuroendocrine neoplasm; nesidioblastoma
Pathology:
- 75-90% of pancreatic neuroendocrine tumors are non-functional
- 10-25% of pancreatic neuroendocrine tumors are functional [1]
- insulinomas ~60% (beta-cell tumors)
- APUDomas (alpha-cell tumors)
- gastrinomas ~20%
- glucagonoma
- somatostatinoma
- VIPoma (vasoactive intestinal polypeptide)
- GRFoma
- CRFoma
- PPoma
- neurotensinoma
- may be benign or malignant
- cannot be determined on basis of histology
Genetics:
- YY1 gene mutation
Clinical manifestations:
1) secretion of gastrointestinal hormones
a) vasoactive intestinal polypeptide (VIP)
1] secretory diarrhea
2] hypokalemia
3] flushing
b) glucagon
1] diabetes mellitus
2] skin rash, especially migrating necrotizing erythema
c) somatostatin
1] gallstones
2] diabetes mellitus
3] steatorrhea
d) gastrin
1] esophagitis
2] peptic ulcer
e) pancreatic polypeptide (P-Poma)
-> no characteristic clinical syndrome
2) secretion of other endocrine peptides
a) GRFoma
b) CRFoma
c) neurotensinoma
d) vasopressinoma
e) MSHoma
3) 80% of tumors are multiple or malignant
Laboratory:
-> serum chemistries
a) serum gastrin
b) serum vasoactive intestinal polypeptide (VIP)
c) serum glucagon
d) serum somatostatin
e) serum pancreatic polypeptide
f) serum chromogranin A may serve as a marker for all APUDomas
Special laboratory:
- endoscopic ultrasonography
Radiology:
1) computed tomography (CT) & spiral CT
2) octreotide radioisotope scan
- most APUDomas express cell surface somatostatin receptors
3) gadolinium-enhanced magnetic resonance imaging (MRI)
4) angiography
Management:
1) treatment is not indicated for asymptomatic patients [5]
2) surgical resection for single & benign tumors
3) high dose proton pump inhibitor for gastrinoma
- omeprazole 40-240 mg/day
4) surgical debulking of tumor
a) VIPoma
b) glucagonoma
c) somatostatinoma
e) P-Poma
4) octreotide injections 50-300 ug SQ QD can control hormonal effects & lead to tumor regression in some patients
5) chemotherapy minimally effective [1]
6) sunitinib & everolimus are active [1]
7) capecitabine plus temozolomide is active [1]
8) chemoembolization can be used to reduce tumor volume in cases of liver metastasis [5]
9) prognosis is better than for adenocarcinoma of the pancreas
Interactions
disease interactions
Related
amine precursor uptake & decarboxylation tumor (APUDoma)
Specific
benign islet cell tumor
CRFoma
glucagonoma
GRFoma; GRHoma
insulinoma
neurotensinoma
pancreatic polypeptideoma (PPoma)
somatostatinoma
Zollinger-Ellison (ZE) syndrome (gastrinoma)
General
carcinoma
neuroendocrine neoplasm
pancreatic neoplasm
References
- Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18, 19.
American College of Physicians, Philadelphia 2009, 2012, 2015, 2018, 2021.
- ARUP Consult: Pancreatic Neuroendocrine Tumors - NET
The Physician's Guide to Laboratory Test Selection & Interpretation
https://arupconsult.com/content/pancreatic-neuroendocrine-tumors
- Davies K, Conlon KC.
Neuroendocrine tumors of the pancreas.
Curr Gastroenterol Rep. 2009 Apr;11(2):119-27.
PMID: 19281699
- Burns WR1, Edil BH.
Neuroendocrine pancreatic tumors: guidelines for management
and update.
Curr Treat Options Oncol. 2012 Mar;13(1):24-34
PMID: 22198808
- Kleynberg RL, Guralnik G
Pancreatic Cancer: Difficult Diagnosis, Ominous Outlook.
Medscape. March 24, 2016
http://reference.medscape.com/features/slideshow/pancreatic-cancer