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Neuroendocrine Neoplasms (Neuroendocrine Tumor [Carcinoid Syndrome])

Marie L Borum, MD, EdD, MPH and Adam Z Horowitz, M.D. Reviewed 05/2023
 


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 NENs are asymptomatic and discovered incidentally during imaging, endoscopy, surgery, or autopsy.

  • Symptoms:

    • Episodic flushing (85%)—clinical hallmark

    • Secretory diarrhea (80%)

    • Broncho...

TREATMENT

  • Surgery is first-line treatment for localized GEP-NENs.

  • Surgical cure is unlikely in the presence of intra-abdominal and widespread metastases (2).

  • Treatment has evolved slowly due to lack of...

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

  • Bhattacharya S, Davar J, Dreyfus G, et al. Carcinoid Heart Disease. Circulation. 2007;116(24).

  • Dasari A, Shen C, Halperin D, et al. Trends ...

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 neoplasms (NENs) is preferred.

  • <10% of NENs produce symptoms, often leading to delays in diagnosis.

  • NENs are often misdiagno...

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