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Neuroendocrine Tumor (Carcinoid Syndrome)

Marie L Borum, MD, EdD, MPH and ChiChi Udochi, B.A., M.D. Reviewed 06/2021
 


BASICS

DESCRIPTION

  • Cluster of symptoms mediated by the release of humoral factors secreted by carcinoid tumors:

    • Cutaneous flushing

    • Diarrhea

    • Bronchospasms

  • The term carcinoid has fallen out of favor due to l...

DIAGNOSIS

HISTORY

  • Most NEN are asymptomatic and discovered incidentally during imaging, endoscopy, surgery, or autopsy.

  • Record timing, frequency, and precipitating factors:

    • Episodic flushing (85%)—clinic...

TREATMENT

Surgery is first-line treatment for local GEP-NET. Surgical cure is unlikely in the presence of intra-abdominal and widespread metastases (2). Treatment has evolved slowly due to lack of un...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Carcinoid tumors often associated with development of a second primary tumor due to tumorigenic activity of bioactive products. Patients with carcinoid tumors of ...

REFERENCES

1
Oberg  K, Couvelard  A, Delle Fave  G, et al; for Antibes Consensus Conference participants. ENETS consensus guidelines for standard of care in neuroendocrine tumou...

ADDITIONAL READING

  • Cives  M, Strosberg  JR. Gastroenteropancreatic neuroendocrine tumors. CA Cancer J Clin.  2018;68(6):471–487. [View Abstract on OvidMedline] ...

CODES

ICD10

  • E34.0 Carcinoid syndrome

  • D3A.8 Other benign neuroendocrine tumors

SNOMED

  • 35868009 Carcinoid syndrome (disorder)

  • 255046005 neuroendocrine tumor (disorder)

CLINICAL PEARLS

  • The term carcinoid has fallen out of favor. Neuroendocrine neoplasia (NEN) is preferred.

  • <10% of NEN produce symptoms, often leading to delay in diagnosis.

  • NEN is often misdiagnosed a...

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