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Bronchial Carcinoid

By

Robert L. Keith

, MD, Division of Pulmonary Sciences and Critial Care Medicine, Department of Medicine, Eastern Colorado VA Healthcare System, University of Colorado

Last full review/revision Jul 2020| Content last modified Jul 2020
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Bronchial carcinoids are rare (1 to 2% of all lung cancers in adults), slow-growing neuroendocrine tumors arising from bronchial mucosa; they affect patients in their 40s to 60s.

Carcinoid tumors develop from neuroendocrine cells in the gastrointestinal tract (90%), pancreas, pulmonary bronchi, and rarely the genitourinary tract. Although carcinoids are often benign or only locally invasive, those affecting the bronchus are frequently malignant . Some carcinoid tumors are endocrinologically active; the likelihood varies by site of origin and is highest for tumors originating in the ileum and proximal colon (40 to 50%) and lower with bronchial carcinoids.

Half of patients with bronchial carcinoid are asymptomatic, and half present with symptoms of airway obstruction, including dyspnea, wheezing, and cough, which often leads to a misdiagnosis of asthma. Recurrent pneumonia, hemoptysis, and chest pain are also common.

Paraneoplastic syndromes, including Cushing syndrome due to ectopic ACTH, acromegaly due to ectopic growth hormone–releasing factor, and Zollinger-Ellison syndrome due to ectopic gastrin production, are more common than carcinoid syndrome, which occurs in < 3% of patients with the tumor.

Carcinoid syndrome symptoms include

  • Flushing

  • Diarrhea

  • Bronchospasm

Chronic sequelae of carcinoid syndrome include

  • Telangiectasias

  • Right-sided valvular heart disease

  • Retroperitoneal fibrosis

A left-sided heart murmur (mitral stenosis or regurgitation) due to serotonin-induced valvular damage occurs rarely with bronchial carcinoids (as opposed to the right-sided valvular lesions of GI carcinoid).

Diagnosis

  • Bronchoscopic biopsy

Diagnosis of bronchial carcinoid is based on bronchoscopic biopsy, but evaluation often initially involves chest CT, which reveals tumor calcifications in up to one third of patients.

Indium-111–labeled octreotide scans are useful for determining regional and metastatic spread.

Increased urinary serotonin and 5-hydroxyindoleacetic acid levels support the diagnosis, but these substances are not commonly elevated.

Treatment

  • Surgery

Treatment of bronchial carcinoid is with surgical removal with or without adjuvant chemotherapy and/or radiation therapy.

Prognosis depends on tumor type. Five-year survival for well-differentiated carcinoids is > 90%; for atypical tumors, it is 50 to 70%.

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