islet cell tumor; pancreatic neuroendocrine neoplasm; nesidioblastoma
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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)
- may be benign or malignant
- cannot be determined on basis of histology
Clinical manifestations
- secretion of gastrointestinal hormones
- vasoactive intestinal polypeptide (VIP)
- glucagon
- diabetes mellitus
- skin rash, especially migrating necrotizing erythema
- somatostatin
- gastrin
- pancreatic polypeptide (P-Poma)
- no characteristic clinical syndrome
- secretion of other endocrine peptides
- 80% of tumors are multiple or malignant
Laboratory
- serum chemistries
- serum gastrin
- serum vasoactive intestinal polypeptide (VIP)
- serum glucagon
- serum somatostatin
- serum pancreatic polypeptide
- serum chromogranin A may serve as a marker for all APUDomas
Diagnostic procedures
Radiology
- computed tomography (CT) & spiral CT
- octreotide radioisotope scan
- most APUDomas express cell surface somatostatin receptors
- gadolinium-enhanced magnetic resonance imaging (MRI)
- angiography
Management
- treatment is not indicated for asymptomatic patients[5]
- surgical resection for single & benign tumors
- high dose proton pump inhibitor for gastrinoma
- omeprazole 40-240 mg/day
- surgical debulking of tumor
- octreotide injections 50-300 ug SQ QD can control hormonal effects & lead to tumor regression in some patients
- chemotherapy minimally effective[1]
- sunitinib & everolimus are active[1]
- capecitabine plus temozolomide is active[1]
- chemoembolization can be used to reduce tumor volume in cases of liver metastasis[5]
- prognosis is better than for adenocarcinoma of the pancreas
More general terms
More specific terms
- benign islet cell tumor
- CRFoma
- glucagonoma
- GRFoma; GRHoma
- insulinoma
- neurotensinoma
- pancreatic polypeptideoma (PPoma)
- somatostatinoma
- Zollinger-Ellison (ZE) syndrome (gastrinoma)
Additional terms
References
- ↑ 1.0 1.1 1.2 1.3 1.4 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: https://www.ncbi.nlm.nih.gov/pubmed/19281699
- ↑ Burns WR1, Edil BH. Neuroendocrine pancreatic tumors: guidelines for management and update. Curr Treat Options Oncol. 2012 Mar;13(1):24-34 PMID: https://www.ncbi.nlm.nih.gov/pubmed/22198808
- ↑ 5.0 5.1 5.2 Kleynberg RL, Guralnik G Pancreatic Cancer: Difficult Diagnosis, Ominous Outlook. Medscape. March 24, 2016 http://reference.medscape.com/features/slideshow/pancreatic-cancer