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Carcinoid Syndrome (Neuroendocrine Tumor)

Bridget K. Caulkins, MD and Matthew K. Hawks, MD Reviewed 06/2018
 


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 carcinoid tumors are asymptomatic and discovered incidentally at imaging, endoscopy, surgery, or autopsy.

  • 20% of well-differentiated NETs will have carcinoid symptoms.

  • Due to nonsp...

TREATMENT

Surgical resection is first line for small and localized tumors and to alleviate any obstructive symptoms. Surgical cure is unlikely in the presence of intra-abdominal and widespread metasta...

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

  • Enzler T, Fojo T. Long-acting somatostatin analogues in the treatment of unresectable/metastatic neuroendocrine tumors. Semin Oncol.  2017;44(2):141–156.

  • Kulke MH, Hörsch D, Cap...

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

  • <10% of carcinoid tumors produce symptoms. This often leads to a delay in diagnosis; often misdiagnosed as IBS

  • Major symptoms are diarrhea, flushing, and wheezing.

  • Diagnosis establish...

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