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neuroendocrine neoplasm

Classification: - pancreatic neuroendocrine tumors - gastrointestinal neuroendocrine tumors Pathology: distribution of neuroendocrine tumors 1) carcinoid tumor (lung, gastrointestinal tract) 2) islet cell tumor (pancreas) 3) ganglioneuroblastoma (see neuroblastoma) 4) neuroblastoma (adrenal, paraspinal) 5) paraganglioma (adrenal, autonomic ganglia associated) - chemodectoma (central nervous system) - pheochromocytoma (adrenal) - glomus jugulare tumor 6) medullary thyroid carcinoma (thyroid) 7) Merkel cell carcinoma (skin) 8) small cell carcinoma (lung) * 25% of neuroendocrine neoplasms produce hormones [2] * most are hormonally non-functioning [2] Genetics: - implicated genes; - MAX, SDHB, SDHC, SDHD, TMEM127, VHL [5] Clinical manifestations: - gastrointesinal neuroendocrine tumors may produce - serotonin resulting in carcinoid syndrome (diarhea & facial flushing) - gastrin resulting in peptic ulcer disease - pancreatic neuroendocrine tumors may produce - insulin - gastrin - glucagon - somatostatin - vasoactive intestinal polypeptide - neuroendocrine tumors may be a component of multiple endocrine neoplasia type 1 (MEN1) - hyperparathyroidism, pituitary tumors, enteropancreatic tumors Laboratory: - neuroendocrine tumor genomic sequencing [5] - neuroendocrine tumor gene duplication & deletion analysis [5] Radiology: - CT imaging with radiolabeled somatostatin analogs - MRI Complications: - non-functioning tumors may be asymptomatic & develop metastatic disease years before diagnosis Management: - well-differentiated neuroendocrine neoplasms are indolent & require only observation & serial imaging [2] - surgical resection for localized tumors [2] - octreotide or lanreotide for tumors with somatostatin receptors - islet cell neuroendocrine tumors (when treatment needed) - temozolamide + capecitabine or sunitinib or everolimus [2] - everolimus has modest activity in GI neuroendcrine tumors - other agents used for islet cell neuroendocrine tumors are not effective [2]

Related

endocrine neoplasm

Specific

amine precursor uptake & decarboxylation tumor (APUDoma) carcinoid (argentaffinoma) islet cell tumor; pancreatic neuroendocrine neoplasm; nesidioblastoma neuroblastoma neuroendocrine carcinoma paraganglioma pheochromocytoma; paroxysmal hypertension; adrenal medullary paraganglioma; chromoffinoma

General

neuroectodermal neoplasm

References

  1. Harrison's Principles of Internal Medicine, 14th ed. Fauci et al (eds), McGraw-Hill Inc. NY, 1998, pg 584
  2. Medical Knowledge Self Assessment Program (MKSAP) 16, 17, 18, 19. American College of Physicians, Philadelphia 2012, 2015, 2018, 2021. - Medical Knowledge Self Assessment Program (MKSAP) 19 Board Basics. An Enhancement to MKSAP19. American College of Physicians, Philadelphia 2022
  3. Reidy DL, Tang LH, Saltz LB. Treatment of advanced disease in patients with well- differentiated neuroendocrine tumors. Nat Clin Pract Oncol. 2009 Mar;6(3):143-52. PMID: 19190591
  4. Kulke MH, Siu LL, Tepper JE et al Future directions in the treatment of neuroendocrine tumors: consensus report of the National Cancer Institute Neuroendocrine Tumor clinical trials planning meeting. J Clin Oncol. 2011 Mar 1;29(7):934-43. PMID: 21263089
  5. CPT :accession 81437, 81438
  6. Guidelines: - 2013 NANETS Consensus Guidelines for the Diagnosis of Neuroendocrine Tumor. https://www.nanets.net/2013-nanets-guidelines - The 2016 ENETS Consensus Guidelines for the Diagnosis and Treatment of Neuroendocrine Tumors. http://www.enets.org/current_guidelines.html - ESMO Clinical Practice Guidelines: Neuroendocrine Tumours. http://www.esmo.org/Guidelines/Neuroendocrine-Tumours
  7. Singh S, Asa SL, Dey C et al Diagnosis and management of gastrointestinal neuroendocrine tumors: An evidence-based Canadian consensus. Cancer Treat Rev. 2016 Jun;47:32-45. Review. PMID: 27236421
  8. Kaderli RM, Spanjol M, Kollar A et al Therapeutic Options for Neuroendocrine Tumors. A Systematic Review and Network Meta-analysis. JAMA Oncol. Published online February 14, 2019 PMID: 30763436 https://jamanetwork.com/journals/jamaoncology/fullarticle/2724799 - Strosberg JR, Al-Toubah T, Cives M Evaluating Risks and Benefits of Evolving Systemic Treatments of Neuroendocrine Tumors. JAMA Oncol. Published online February 14, 2019 PMID: 30763444 https://jamanetwork.com/journals/jamaoncology/fullarticle/2724795