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Thymic neuroendocrine tumor

MedGen UID:
1842737
Concept ID:
C5681534
Neoplastic Process
Synonyms: neuroendocrine neoplasm of thymus; thymic neuroendocrine tumor; thymus NET; thymus neuroendocrine neoplasm; thymus neuroendocrine tumor; thymus neuroendocrine tumor, well differentiated, low or intermediate grade; thymus neuroendocrine tumour
 
Monarch Initiative: MONDO:0019964
Orphanet: ORPHA97289

Definition

A rare, malignant, primary thymic neoplasm originating from neuroendocrine cells, presenting as a mass within the anterior mediastinum. Patients typically present with nonspecific symptoms, such as chest pain, cough, shortness of breath, or in some cases, superior vena cava syndrome, although patients could be asymptomatic during the early stages or present with multiple endocrine neoplasia type I. Ectopic production of ACTH and serotonin can lead to Cushing syndrome and carcinoid syndrome, respectively. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThymic neuroendocrine tumor

Professional guidelines

PubMed

Yokoi K, Kondo K, Fujimoto K, Hara M, Kadota Y, Kawaguchi K, Kunitoh H, Matsuno Y, Nakajima J, Nishio M, Ogawa K, Omasa M
Jpn J Clin Oncol 2017 Dec 1;47(12):1119-1122. doi: 10.1093/jjco/hyx138. PMID: 29036455

Recent clinical studies

Etiology

Suwal S, Chen YY, Huang SD, Li WF, Chen H
Curr Med Imaging 2024;20:1-8. doi: 10.2174/0115734056259584231112105510. PMID: 38389359
Marom EM, Fang W, Ruffini E, Detterbeck F, Ahmad U, Appel S, Bille A, Boubia S, Brambilla C, Cilento V, Cangir AK, Falkson C, Filosso PL, Giaccone G, Girard N, Goren E, Guerrera F, Huang J, Infante M, Kim DK, Lucchi M, Marino M, Nicholson AG, Okumura M, Rami-Porta R, Rimner A, Simone CB 2nd, Asamura H; Members of the IASLC Staging and Prognostic Factors Committee and of the Advisory Boards and Participating Institutions
J Thorac Oncol 2023 Dec;18(12):1672-1688. Epub 2023 Sep 9 doi: 10.1016/j.jtho.2023.09.001. PMID: 37689390
Sakane T, Sakamoto Y, Masaki A, Murase T, Okuda K, Nakanishi R, Inagaki H
Clin Lung Cancer 2021 Mar;22(2):92-99.e4. Epub 2020 Dec 2 doi: 10.1016/j.cllc.2020.11.010. PMID: 33414055
Hsu CH, Chan JK, Yin CH, Lee CC, Chern CU, Liao CI
PLoS One 2019;14(12):e0227197. Epub 2019 Dec 31 doi: 10.1371/journal.pone.0227197. PMID: 31891634Free PMC Article
Wood K, Byron E, Janisch L, Salgia R, Sharma MR
Am J Clin Oncol 2018 Oct;41(10):963-966. doi: 10.1097/COC.0000000000000400. PMID: 28654574Free PMC Article

Diagnosis

Suwal S, Chen YY, Huang SD, Li WF, Chen H
Curr Med Imaging 2024;20:1-8. doi: 10.2174/0115734056259584231112105510. PMID: 38389359
Marx A, Chan JKC, Chalabreysse L, Dacic S, Detterbeck F, French CA, Hornick JL, Inagaki H, Jain D, Lazar AJ, Marino M, Marom EM, Moreira AL, Nicholson AG, Noguchi M, Nonaka D, Papotti MG, Porubsky S, Sholl LM, Tateyama H, Thomas de Montpréville V, Travis WD, Rajan A, Roden AC, Ströbel P
J Thorac Oncol 2022 Feb;17(2):200-213. Epub 2021 Oct 22 doi: 10.1016/j.jtho.2021.10.010. PMID: 34695605
Pan J, Lin W, Zong L, Wen J, Jiang F, Chen G
Ann Palliat Med 2022 Apr;11(4):1575-1581. Epub 2021 Jun 25 doi: 10.21037/apm-21-86. PMID: 34263608
Okumura T, Takayama S, Nishio SI, Miyakoshi T, Noguchi T, Kobayashi T, Fukushima T, Sekiguchi N, Otsuki T, Komatsu M, Koizumi T
Thorac Cancer 2019 Jul;10(7):1648-1653. Epub 2019 Jun 11 doi: 10.1111/1759-7714.13099. PMID: 31187563Free PMC Article
Li Y, Peng Y, Jiang X, Cheng Y, Zhou W, Su T, Xie J, Zhong X, Song D, Wu L, Fan L, Li M, Hong J, Wang W, Ning G, Cao Y
Eur J Endocrinol 2017 Feb;176(2):187-194. Epub 2016 Nov 15 doi: 10.1530/EJE-16-0546. PMID: 27913610

Therapy

Reynel M, Villegas Y, Werthmann PG, Kiene H, Kienle GS
Medicine (Baltimore) 2020 Jan;99(5):e18990. doi: 10.1097/MD.0000000000018990. PMID: 32000435Free PMC Article
Cottereau AS, Bricaire L, Arrondeau J, Dechmi A, Montravers F, Coriat R, Clerc J, Groussin L, Tenenbaum F
Invest New Drugs 2020 Aug;38(4):1196-1199. Epub 2019 Oct 24 doi: 10.1007/s10637-019-00865-6. PMID: 31650444
Okumura T, Takayama S, Nishio SI, Miyakoshi T, Noguchi T, Kobayashi T, Fukushima T, Sekiguchi N, Otsuki T, Komatsu M, Koizumi T
Thorac Cancer 2019 Jul;10(7):1648-1653. Epub 2019 Jun 11 doi: 10.1111/1759-7714.13099. PMID: 31187563Free PMC Article
Wang X, Li Y, Duan J, Chen Y, Yuan B, Qi Z, Tan H
Oncologist 2019 Jun;24(6):798-802. Epub 2018 Nov 9 doi: 10.1634/theoncologist.2018-0291. PMID: 30413666Free PMC Article
Wood K, Byron E, Janisch L, Salgia R, Sharma MR
Am J Clin Oncol 2018 Oct;41(10):963-966. doi: 10.1097/COC.0000000000000400. PMID: 28654574Free PMC Article

Prognosis

Marom EM, Fang W, Ruffini E, Detterbeck F, Ahmad U, Appel S, Bille A, Boubia S, Brambilla C, Cilento V, Cangir AK, Falkson C, Filosso PL, Giaccone G, Girard N, Goren E, Guerrera F, Huang J, Infante M, Kim DK, Lucchi M, Marino M, Nicholson AG, Okumura M, Rami-Porta R, Rimner A, Simone CB 2nd, Asamura H; Members of the IASLC Staging and Prognostic Factors Committee and of the Advisory Boards and Participating Institutions
J Thorac Oncol 2023 Dec;18(12):1672-1688. Epub 2023 Sep 9 doi: 10.1016/j.jtho.2023.09.001. PMID: 37689390
Shin DW, Cho JH, Ha J, Jung KW
J Thorac Oncol 2022 Jun;17(6):827-837. Epub 2022 Feb 11 doi: 10.1016/j.jtho.2022.02.001. PMID: 35158083
Sakane T, Sakamoto Y, Masaki A, Murase T, Okuda K, Nakanishi R, Inagaki H
Clin Lung Cancer 2021 Mar;22(2):92-99.e4. Epub 2020 Dec 2 doi: 10.1016/j.cllc.2020.11.010. PMID: 33414055
Wood K, Byron E, Janisch L, Salgia R, Sharma MR
Am J Clin Oncol 2018 Oct;41(10):963-966. doi: 10.1097/COC.0000000000000400. PMID: 28654574Free PMC Article
Li Y, Peng Y, Jiang X, Cheng Y, Zhou W, Su T, Xie J, Zhong X, Song D, Wu L, Fan L, Li M, Hong J, Wang W, Ning G, Cao Y
Eur J Endocrinol 2017 Feb;176(2):187-194. Epub 2016 Nov 15 doi: 10.1530/EJE-16-0546. PMID: 27913610

Clinical prediction guides

Suwal S, Chen YY, Huang SD, Li WF, Chen H
Curr Med Imaging 2024;20:1-8. doi: 10.2174/0115734056259584231112105510. PMID: 38389359
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
Zheng H, Zhou S, Tang W, Wang Q, Zhang X, Jin X, Yuan Y, Fu J
BMC Neurol 2020 Mar 4;20(1):76. doi: 10.1186/s12883-020-01659-7. PMID: 32126984Free PMC Article
Li Y, Peng Y, Jiang X, Cheng Y, Zhou W, Su T, Xie J, Zhong X, Song D, Wu L, Fan L, Li M, Hong J, Wang W, Ning G, Cao Y
Eur J Endocrinol 2017 Feb;176(2):187-194. Epub 2016 Nov 15 doi: 10.1530/EJE-16-0546. PMID: 27913610
Fukai I, Masaoka A, Fujii Y, Yamakawa Y, Yokoyama T, Murase T, Eimoto T
Ann Thorac Surg 1999 Jan;67(1):208-11. doi: 10.1016/s0003-4975(98)01063-7. PMID: 10086551

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