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Bronchopulmonary neuroendocrine tumors
Background
Overview
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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Risk factors
Risk factors for bronchopulmonary NETs are not fully understood. However, certain chronic lung diseases, exposure to environmental toxins, and genetic predisposition may contribute to the development of these tumors. The presence of neuroendocrine cell hyperplasia, tumorlets, or diffuse idiopathic neuroendocrine cell hyperplasia can suggest the development of multiple NETs.
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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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Guidelines
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 European Hip Society (EHS/EAU/ISTH/EACTAIC/EACTS/AATS/ESTS/EBCOG/EKS/ESAIC/NATA/SRLF/EURAPS 2024), 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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Screening and diagnosis
Indications for screening
As per ES 2012 guidelines:
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.
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Diagnosis
Classification and risk stratification
Diagnostic investigations
Diagnostic imaging: as per ENETS 2015 guidelines, obtain contrast-enhanced chest CT for the diagnosis of bronchopulmonary NETs.
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consider obtaining HRCT if contrast administration is contraindicated. B
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Laboratory tests
Evaluation for MEN1
Imaging for staging
Diagnostic procedures
Diagnostic bronchoscopy
As per ENETS 2015 guidelines:
Consider performing a bronchoscopy, preferably flexible bronchoscopy, for preoperative staging and assessment of central airway tumors.
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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.
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Biopsy and histopathology
Medical management
Management of local/locoregional disease, watchful waiting: as per ESMO 2021 guidelines, 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 or MEN1, 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
Perioperative care
Preoperative cardiorespiratory evaluation
As per ENETS 2015 guidelines:
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.
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Perioperative thromboprophylaxis (lobectomy/segmentectomy)
Perioperative thromboprophylaxis (pneumonectomy)
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.
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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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