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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) 9) appendix is the most common site of well-differentiated neuroendocrine neoplasms * 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 if > 2 cm [2,9] - 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 - Medical Knowledge Self Assessment Program (MKSAP) 20 American College of Physicians, Philadelphia 2025
  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
  9. Nesti C, Brautigam K, Benavent M, et al. Hemicolectomy versus appendectomy for patients with appendiceal neuroendocrine tumours 1-2 cm in size: a retrospective, Europe-wide, pooled cohort study. Lancet Oncol. 2023;24:187-94. PMID: 36640790
  10. NCCN Clinical Guidelines in Oncology. Neuroendocrine and adrenal tumors. Version 2. 2022. NCCN.org. https://www.nccn.org/guidelines/guidelines-detail?category=1&id=1448