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Bronchial endocrine tumor

MedGen UID:
977627
Concept ID:
CN294250
Disease or Syndrome
Synonyms: bronchial endocrine tumor; Bronchial NET; bronchial NET; bronchial neuroendocrine tumor; Bronchial neuroendocrine tumor; bronchial neuroendocrine tumour; bronchus NET; bronchus neuroendocrine neoplasm; bronchus neuroendocrine tumor; bronchus neuroendocrine tumor, well differentiated, low or intermediate grade; bronchus neuroendocrine tumour; neuroendocrine neoplasm of bronchus
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019963
Orphanet: ORPHA97287

Definition

A rare neuroendocrine neoplasm characterized by origin from pulmonary neuroendocrine cells and ranging from low-grade typical carcinoid and intermediate-grade atypical carcinoid to high-grade large-cell neuroendocrine carcinoma and small-cell carcinoma. Two thirds of the tumors are located in the major bronchi, with a predilection for the right lung, in particular the middle lobe. Most patients with central bronchial tumors present with hemoptysis, cough, recurrent pulmonary infections, fever, chest discomfort, and unilateral wheezing, while peripheral carcinoids are usually discovered only incidentally. Carcinoid syndrome or Cushing syndrome are very rare. The tumors may be part of multiple endocrine neoplasia type 1. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBronchial endocrine tumor

Professional guidelines

PubMed

Pavel M, O'Toole D, Costa F, Capdevila J, Gross D, Kianmanesh R, Krenning E, Knigge U, Salazar R, Pape UF, Öberg K; Vienna Consensus Conference participants
Neuroendocrinology 2016;103(2):172-85. Epub 2016 Jan 5 doi: 10.1159/000443167. PMID: 26731013
Norton JA, Krampitz G, Jensen RT
Surg Oncol Clin N Am 2015 Oct;24(4):795-832. Epub 2015 Jul 27 doi: 10.1016/j.soc.2015.06.008. PMID: 26363542Free PMC Article
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366

Recent clinical studies

Etiology

Ragnarsson O, Juhlin CC, Torpy DJ, Falhammar H
Trends Endocrinol Metab 2024 Apr;35(4):347-360. Epub 2023 Dec 23 doi: 10.1016/j.tem.2023.12.003. PMID: 38143211
Faggiano A, Fazzalari B, Mikovic N, Russo F, Zamponi V, Mazzilli R, Guarnieri V, Piane M, Visco V, Petrucci S
Genes (Basel) 2023 Sep 10;14(9) doi: 10.3390/genes14091782. PMID: 37761922Free PMC Article
Dromain C, Vullierme MP, Hicks RJ, Prasad V, O'Toole D, de Herder WW, Pavel M, Faggiano A, Kos-Kudla B, Öberg K, Krejs GJ, Grande E, Niederle B, Sundin A
J Neuroendocrinol 2022 Mar;34(3):e13044. Epub 2021 Oct 25 doi: 10.1111/jne.13044. PMID: 34693574Free PMC Article
Brabander T, van der Zwan WA, Teunissen JJM, Kam BLR, Feelders RA, de Herder WW, van Eijck CHJ, Franssen GJH, Krenning EP, Kwekkeboom DJ
Clin Cancer Res 2017 Aug 15;23(16):4617-4624. Epub 2017 Apr 20 doi: 10.1158/1078-0432.CCR-16-2743. PMID: 28428192
Norton JA, Krampitz G, Jensen RT
Surg Oncol Clin N Am 2015 Oct;24(4):795-832. Epub 2015 Jul 27 doi: 10.1016/j.soc.2015.06.008. PMID: 26363542Free PMC Article

Diagnosis

Ragnarsson O, Juhlin CC, Torpy DJ, Falhammar H
Trends Endocrinol Metab 2024 Apr;35(4):347-360. Epub 2023 Dec 23 doi: 10.1016/j.tem.2023.12.003. PMID: 38143211
Norton JA, Krampitz G, Jensen RT
Surg Oncol Clin N Am 2015 Oct;24(4):795-832. Epub 2015 Jul 27 doi: 10.1016/j.soc.2015.06.008. PMID: 26363542Free PMC Article
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366
Isidori AM, Lenzi A
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1217-25. doi: 10.1590/s0004-27302007000800007. PMID: 18209859
Wajchenberg BL, Mendonça B, Liberman B, Adelaide M, Pereira A, Kirschner MA
J Steroid Biochem Mol Biol 1995 Jun;53(1-6):139-51. doi: 10.1016/0960-0760(95)00044-z. PMID: 7626446

Therapy

Duke ES, Bradford D, Marcovitz M, Amatya AK, Mishra-Kalyani PS, Nguyen E, Price LSL, Fourie Zirkelbach J, Li Y, Bi Y, Kraft J, Dorff SE, Scepura B, Stephenson M, Ojofeitimi I, Nair A, Han Y, Tezak Z, Lemery SJ, Pazdur R, Larkins E, Singh H
Clin Cancer Res 2023 Sep 15;29(18):3573-3578. doi: 10.1158/1078-0432.CCR-23-0459. PMID: 37265412Free PMC Article
Baudin E, Hayes AR, Scoazec JY, Filosso PL, Lim E, Kaltsas G, Frilling A, Chen J, Kos-Kudła B, Gorbunova V, Wiedenmann B, Nieveen van Dijkum E, Ćwikła JB, Falkerby J, Valle JW, Kulke MH, Caplin ME; ENETS 2016 Munich Advisory Board Participants; ENETS 2016 Munich Advisory Board Participants
Neuroendocrinology 2019;108(1):7-17. Epub 2018 Sep 24 doi: 10.1159/000493980. PMID: 30248673
Brabander T, van der Zwan WA, Teunissen JJM, Kam BLR, Feelders RA, de Herder WW, van Eijck CHJ, Franssen GJH, Krenning EP, Kwekkeboom DJ
Clin Cancer Res 2017 Aug 15;23(16):4617-4624. Epub 2017 Apr 20 doi: 10.1158/1078-0432.CCR-16-2743. PMID: 28428192
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366
Cortes JE, Pazdur R
J Clin Oncol 1995 Oct;13(10):2643-55. doi: 10.1200/JCO.1995.13.10.2643. PMID: 7595719

Prognosis

Faggiano A, Fazzalari B, Mikovic N, Russo F, Zamponi V, Mazzilli R, Guarnieri V, Piane M, Visco V, Petrucci S
Genes (Basel) 2023 Sep 10;14(9) doi: 10.3390/genes14091782. PMID: 37761922Free PMC Article
Araujo-Castro M
Endocrinol Diabetes Nutr (Engl Ed) 2023 Mar;70 Suppl 1:63-73. Epub 2022 Nov 14 doi: 10.1016/j.endien.2022.11.014. PMID: 36396595
Baudin E, Hayes AR, Scoazec JY, Filosso PL, Lim E, Kaltsas G, Frilling A, Chen J, Kos-Kudła B, Gorbunova V, Wiedenmann B, Nieveen van Dijkum E, Ćwikła JB, Falkerby J, Valle JW, Kulke MH, Caplin ME; ENETS 2016 Munich Advisory Board Participants; ENETS 2016 Munich Advisory Board Participants
Neuroendocrinology 2019;108(1):7-17. Epub 2018 Sep 24 doi: 10.1159/000493980. PMID: 30248673
Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366
Cortes JE, Pazdur R
J Clin Oncol 1995 Oct;13(10):2643-55. doi: 10.1200/JCO.1995.13.10.2643. PMID: 7595719

Clinical prediction guides

Gerlach L, Beyer AL, Kaemmerer D, Sänger J, Evert K, Schulz S, Lupp A
Sci Rep 2023 Nov 3;13(1):18993. doi: 10.1038/s41598-023-46395-3. PMID: 37923782Free PMC Article
Faggiano A, Fazzalari B, Mikovic N, Russo F, Zamponi V, Mazzilli R, Guarnieri V, Piane M, Visco V, Petrucci S
Genes (Basel) 2023 Sep 10;14(9) doi: 10.3390/genes14091782. PMID: 37761922Free PMC Article
Simonds WF
Front Endocrinol (Lausanne) 2023;14:1183297. Epub 2023 Jun 20 doi: 10.3389/fendo.2023.1183297. PMID: 37409236Free PMC Article
Raue F, Frank-Raue K
Recent Results Cancer Res 2015;204:207-25. doi: 10.1007/978-3-319-22542-5_10. PMID: 26494391
Cortes JE, Pazdur R
J Clin Oncol 1995 Oct;13(10):2643-55. doi: 10.1200/JCO.1995.13.10.2643. PMID: 7595719

Recent systematic reviews

Caplin ME, Baudin E, Ferolla P, Filosso P, Garcia-Yuste M, Lim E, Oberg K, Pelosi G, Perren A, Rossi RE, Travis WD; ENETS consensus conference participants
Ann Oncol 2015 Aug;26(8):1604-20. Epub 2015 Feb 2 doi: 10.1093/annonc/mdv041. PMID: 25646366
Isidori AM, Lenzi A
Arq Bras Endocrinol Metabol 2007 Nov;51(8):1217-25. doi: 10.1590/s0004-27302007000800007. PMID: 18209859

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