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Bronchopulmonary neuroendocrine tumors

Definition
Bronchopulmonary NETs are a spectrum of tumors arising from neuroendocrine cells of the bronchopulmonary epithelium.
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Pathophysiology
The pathophysiology of bronchopulmonary NETs is not fully understood, but they are known to arise from neuroendocrine cells. Some occur sporadically, while others are associated with genetic syndromes, such as MEN1 and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia.
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Epidemiology
The incidence of bronchopulmonary carcinoids (typical and atypical) in the US is estimated at 1.57 per 100,000.
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Disease course
Bronchopulmonary NETs are a diverse group of neoplasms, namely, typical carcinoid tumor, atypical carcinoid tumor, large-cell neuroendocrine carcinoma, and small-cell lung carcinoma, that can present with a variety of clinical features. Common symptoms include cough, hemoptysis, and wheezing, although some patients may be asymptomatic. In some cases, these tumors can present as ramifying bronchial tumors following the bronchial tree.
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Prognosis and risk of recurrence
The prognosis of bronchopulmonary NETs can vary depending on several factors. The 1-, 3-, and 5-year survival rates are estimated at 92.7%, 80.3%, and 71.9%, respectively.
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Key sources
The following summarized guidelines for the evaluation and management of bronchopulmonary neuroendocrine tumors are prepared by our editorial team based on guidelines from the American Association for Thoracic Surgery (AATS/ESTS 2022), the European Society of Medical Oncology (ESMO 2021), the European Neuroendocrine Tumor Society (ENETS 2015), the American College of Chest Physicians (ACCP 2013), and the Endocrine Society (ES 2012).
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Guidelines

1.Screening and diagnosis

Indications for screening
Obtain chest CT or MRI every 1-2 years to detect bronchopulmonary carcinoid tumors in patients with MEN1.
B
Do not obtain biochemical evaluation with urinary 5-HIAA or chromogranin A.
D
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  • Diagnosis

2.Classification and risk stratification

Prognostic assessment: use the WHO and pTNM classifications for prognostic classification.
B
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3.Diagnostic investigations

Diagnostic imaging: obtain contrast-enhanced chest CT for the diagnosis of bronchopulmonary NETs.
B
consider obtaining HRCT if contrast administration is contraindicated.
B
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  • Laboratory tests

  • Evaluation for MEN1

  • Imaging for staging

4.Diagnostic procedures

Diagnostic bronchoscopy
Consider performing a bronchoscopy, preferably flexible bronchoscopy, for preoperative staging and assessment of central airway tumors.
B
Consider preferring rigid bronchoscopy for obtaining biopsy specimens in patients at high risk for bleeding.
C
Insufficient evidence regarding the added value of new bronchoscopic techniques to increase the sensitivity of detection of primary tumors or recurrence.
I

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  • Biopsy and histopathology

5.Medical management

Management of local/locoregional disease, watchful waiting, ESMO: consider offering initial imaging follow-up without treatment to determine the tumor growth rate in patients with cT1N0 lung carcinoid within the setting of diffuse pulmonary neuroendocrine cell hyperplasia orMEN1 , multifocal primaries, or comorbid conditions.
C

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  • Management of local/locoregional disease (surgery and locoregional procedures)

  • Management of local/locoregional disease (systemic therapy)

  • Management of advanced/metastatic disease (watchful waiting)

  • Management of advanced/metastatic disease (systemic therapy)

  • Management of advanced/metastatic disease (surgery and locoregional procedures)

  • Management of functioning syndromes

6.Perioperative care

Preoperative cardiorespiratory evaluation
Obtain functional respiratory tests to assess the surgical risk and the association with chronic obstructive airway disease and to screen for bronchostenosis.
B
Obtain echocardiography in patients with carcinoid syndrome. Assess left and right-side valves in patients with pulmonary carcinoids.
B

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  • Perioperative thromboprophylaxis (lobectomy/segmentectomy)

  • Perioperative thromboprophylaxis (pneumonectomy)

7.Follow-up and surveillance

Follow-up: as per ESMO 2021 guidelines, obtain life-long follow-up in patients with lung carcinoids since recurrences remain very common.
B
obtain life-long follow-up with low-radiation imaging and increasing interval of time, adjusted to prognostic factors, after radical resection of lung carcinoids.
B
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