Peripheral pulmonary carcinoid tumor
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At the time the article was created Yuranga Weerakkody had no recorded disclosures.
View Yuranga Weerakkody's current disclosuresAt the time the article was last revised Alexandru Calciu had no financial relationships to ineligible companies to disclose.
View Alexandru Calciu's current disclosures- Peripheral pulmonary carcinoid tumours
- Peripheral pulmonary carcinoid tumor
- Peripheral pulmonary carcinoid tumors
Peripheral pulmonary carcinoid tumor refers to a subtype of pulmonary carcinoid tumors that arise within the periphery of the lung. They are considered less common than the more centrally-located bronchial carcinoid tumors.
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Clinical presentation
Many patients tend to be asymptomatic 2. Presentation with carcinoid syndrome is extremely rare 6.
Pathology
Peripheral pulmonary carcinoid tumors are considered a neuroendocrine tumor of the lung. They can be:
typical pulmonary carcinoid tumors (well-differentiated, common)
atypical pulmonary carcinoid tumors (more aggressive, uncommon)
Risk factors
smoking: the rate of carcinoid tumors is similar between smokers and non-smokers, although there is an increased incidence of atypical subtype amongst smokers 5,6
Radiographic features
CT
Most are discovered as an incidental rounded solitary pulmonary nodule. The size at diagnosis can vary but is usually reported to be in the range of 10-30 mm 2. Many have a lobulated margin with an average Hounsfield value on post-contrast imaging of ~50 2. Imaging features are often non-specific and tissue diagnosis is essential in determining diagnosis.
Most peripheral carcinoid tumors tend to involve a subsegmental bronchus 2.
Calcification may be present in a small percentage (~11% ) of tumors 2.
Some authors have suggested a "bronchial triangle sign" which may assist in diagnosis 12.
Nuclear medicine
FDG-PET
May have a sensitivity of around 75% 7. Although most cases will show uptake on an 18-FDG PET, up to a quarter of false-negative has been described 2.
68Ga-Octreotide-PET / 68Ga-DOTATATE
Usually avid and useful for diagnosis 8.
Treatment and prognosis
Some authors consider these as complex tumors, requiring a multidisciplinary approach and long-term follow-up 10.
Differential diagnosis
In some situations for CT appearances consider:
enlarged intrapulmonary / bronchopulmonary node (e.g. station 12,13,14)
For a broader differential see differentials for a solitary pulmonary nodule.
References
- 1. Fink G, Krelbaum T, Yellin A et-al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest. 01;119 (6): 1647-51. doi:10.1378/chest.119.6.1647 - Pubmed citation
- 2. Meisinger QC, Klein JS, Butnor KJ et-al. CT features of peripheral pulmonary carcinoid tumors. AJR Am J Roentgenol. 2011;197 (5): 1073-80. AJR Am J Roentgenol (full text) - doi:10.2214/AJR.10.5954 - Pubmed citation
- 3. Magid D, Siegelman SS, Eggleston JC et-al. Pulmonary carcinoid tumors: CT assessment. J Comput Assist Tomogr. 1989;13 (2): 244-7. Pubmed citation
- 4. Steinfort DP, Finlay M, Irving LB. Diagnosis of peripheral pulmonary carcinoid tumor using endobronchial ultrasound. Ann Thorac Med. 2008;3 (4): 146-8. Ann Thorac Med (full text) - doi:10.4103/1817-1737.43082 - Free text at pubmed - Pubmed citation
- 5. Ducrocq X, Thomas P, Massard G et-al. Operative risk and prognostic factors of typical bronchial carcinoid tumors. Ann. Thorac. Surg. 1998;65 (5): 1410-4. Pubmed citation
- 6. Fink G, Krelbaum T, Yellin A et-al. Pulmonary carcinoid: presentation, diagnosis, and outcome in 142 cases in Israel and review of 640 cases from the literature. Chest. 01;119 (6): 1647-51. doi:10.1378/chest.119.6.1647 - Pubmed citation
- 7. Tanaka A, Akamatsu H, Kawabata H, Ariyasu H, Nakamura Y, Yamamoto N. Peripheral pulmonary carcinoid tumor diagnosed by endobronchial-ultrasound-guided bronchoscopy. Respirology case reports. 4 (1): 10-2. doi:10.1002/rcr2.139 - Pubmed
- 8. Chong S, Lee KS, Chung MJ, Han J, Kwon OJ, Kim TS. Neuroendocrine tumors of the lung: clinical, pathologic, and imaging findings. Radiographics : a review publication of the Radiological Society of North America, Inc. 26 (1): 41-57; discussion 57-8. doi:10.1148/rg.261055057 - Pubmed
- 9. Bhatia K, Ellis S. Unusual lung tumours: an illustrated review of CT features suggestive of this diagnosis. Cancer imaging : the official publication of the International Cancer Imaging Society. 6: 72-82. doi:10.1102/1470-7330.2006.0013 - Pubmed
- 10 .Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD. Pulmonary neuroendocrine (carcinoid) tumors: European Neuroendocrine Tumor Society expert consensus and recommendations for best practice for typical and atypical pulmonary carcinoids. (2015) Annals of oncology : official journal of the European Society for Medical Oncology. 26 (8): 1604-20. doi:10.1093/annonc/mdv041 - Pubmed
- 11. Hendifar AE, Marchevsky AM, Tuli R. Neuroendocrine Tumors of the Lung: Current Challenges and Advances in the Diagnosis and Management of Well-Differentiated Disease. (2017) Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer. 12 (3): 425-436. doi:10.1016/j.jtho.2016.11.2222 - Pubmed
- 12. Coruh A, Kul M, Kuru Öz D et al. Is It Possible to Discriminate Pulmonary Carcinoids from Hamartomas Based on CT Features? Clin Imaging. 2020;62:49-56. doi:10.1016/j.clinimag.2020.02.001 - Pubmed
- 13. Piro R, Tonelli R, Taddei S et al. Atypical Diagnosis for Typical Lung Carcinoid. BMC Pulm Med. 2019;19(1):168. doi:10.1186/s12890-019-0929-0 - Pubmed
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