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Neuroendocrine Tumor (Carcinoid Syndrome)

Samata Pathireddy, MD, Narothama Reddy Aeddula, MD, FACP, FASN and Mathura Babu, MD Reviewed 06/2019
 


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...

DIAGNOSIS

HISTORY

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

  • Record timing, frequency, and precipitating factors:

    • Episodic flushing (85%)—clin...

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 uni...

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 tumours: biochemical markers. Ne...

ADDITIONAL READING

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

  • Enzler T, Fojo T. Long-acting somatost...

CODES

ICD10

  • E34.0 Carcinoid syndrome

  • D3A.8 Other benign neuroendocrine tumors

ICD9

  • 259.2 Carcinoid syndrome

  • 209.60 Benign carcinoid tumor of unknown primary site

SNOMED

  • 35868009 Carcinoid syndrome (disorder)

  • ...

CLINICAL PEARLS

  • The term carcinoid has fallen out of favor. NET is preferred.

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

  • NET is often misdiagnosed as IBS.

  • Major symptoms are di...

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