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Thoracic NUT Carcinoma

MedGen UID:
1646468
Concept ID:
C4684820
Neoplastic Process
Synonym: Thoracic NMC

Definition

A highly aggressive, poorly differentiated carcinoma that arises from the thoracic structures. It is characterized by mutations and rearrangement of the NUT gene. It usually presents at an advanced stage with pleuritic chest pain and pleural effusion, non-productive cough, shortness of breath, and weigh loss. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThoracic NUT Carcinoma

Professional guidelines

PubMed

Lee MS, An S, Song JY, Sung M, Jung K, Chang ES, Choi J, Oh DY, Jeon YK, Yang H, Lakshmi C, Park S, Han J, Lee SH, Choi YL
Cancer Res Treat 2023 Apr;55(2):452-467. Epub 2022 Oct 14 doi: 10.4143/crt.2022.910. PMID: 36265509Free PMC Article
Xie XH, Wang LQ, Qin YY, Lin XQ, Xie ZH, Liu M, Zhang JX, Ouyang M, Liu J, Gu YY, Li SY, Zhou CZ
Orphanet J Rare Dis 2020 Jul 10;15(1):183. doi: 10.1186/s13023-020-01449-x. PMID: 32650830Free PMC Article
French CA
Pathol Int 2018 Nov;68(11):583-595. Epub 2018 Oct 26 doi: 10.1111/pin.12727. PMID: 30362654

Recent clinical studies

Etiology

Ramesh U, Contrera KJ, Shakibai N, Su SY, Brahimaj B, Roberts D, Williams MD, Ferrarotto R, Phan J, Gunn GB, Raza S, DeMonte F, Hanna EY
Head Neck 2024 Jan;46(1):29-36. Epub 2023 Oct 18 doi: 10.1002/hed.27553. PMID: 37853958
Carter T, Crook M, Murch A, Beesley AH, de Klerk N, Charles A, Kees UR
BMC Cancer 2021 Jun 27;21(1):740. doi: 10.1186/s12885-021-08432-0. PMID: 34176480Free PMC Article
Xie XH, Wang LQ, Qin YY, Lin XQ, Xie ZH, Liu M, Zhang JX, Ouyang M, Liu J, Gu YY, Li SY, Zhou CZ
Orphanet J Rare Dis 2020 Jul 10;15(1):183. doi: 10.1186/s13023-020-01449-x. PMID: 32650830Free PMC Article
Lemelle L, Pierron G, Fréneaux P, Huybrechts S, Spiegel A, Plantaz D, Julieron M, Dumoucel S, Italiano A, Millot F, Le Tourneau C, Leverger G, Chastagner P, Carton M, Orbach D
Pediatr Blood Cancer 2017 Dec;64(12) Epub 2017 Jun 23 doi: 10.1002/pbc.26693. PMID: 28643357
Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JHM, Beasley MB, Chirieac LR, Dacic S, Duhig E, Flieder DB, Geisinger K, Hirsch FR, Ishikawa Y, Kerr KM, Noguchi M, Pelosi G, Powell CA, Tsao MS, Wistuba I; WHO Panel
J Thorac Oncol 2015 Sep;10(9):1243-1260. doi: 10.1097/JTO.0000000000000630. PMID: 26291008

Diagnosis

Yoshida A
Histopathology 2024 Jan;84(1):86-101. Epub 2023 Oct 24 doi: 10.1111/his.15063. PMID: 37873676
Lantuejoul S, Pissaloux D, Ferretti GR, McLeer A
Semin Diagn Pathol 2021 Sep;38(5):72-82. Epub 2021 Jun 16 doi: 10.1053/j.semdp.2021.06.005. PMID: 34176698
Chatzopoulos K, Boland JM
Virchows Arch 2021 Jan;478(1):21-30. Epub 2021 Jan 6 doi: 10.1007/s00428-020-03011-3. PMID: 33409598
French CA
Pathol Int 2018 Nov;68(11):583-595. Epub 2018 Oct 26 doi: 10.1111/pin.12727. PMID: 30362654
Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JHM, Beasley MB, Chirieac LR, Dacic S, Duhig E, Flieder DB, Geisinger K, Hirsch FR, Ishikawa Y, Kerr KM, Noguchi M, Pelosi G, Powell CA, Tsao MS, Wistuba I; WHO Panel
J Thorac Oncol 2015 Sep;10(9):1243-1260. doi: 10.1097/JTO.0000000000000630. PMID: 26291008

Therapy

Luo J, Sanchez M, Lee E, Hertzler H, Luong N, Mazzola E, Finstein B, Tamen R, Brisbane G, Nguyen T, Paik PK, Chaft JE, Cheng ML, Khalil H, Piha-Paul SA, Sholl LM, Nishino M, Jänne PA, DuBois SG, Hanna GJ, Shapiro GI, French CA
J Thorac Oncol 2024 May;19(5):829-838. Epub 2023 Dec 27 doi: 10.1016/j.jtho.2023.12.022. PMID: 38154515Free PMC Article
Xie XH, Wang LQ, Qin YY, Lin XQ, Xie ZH, Liu M, Zhang JX, Ouyang M, Liu J, Gu YY, Li SY, Zhou CZ
Orphanet J Rare Dis 2020 Jul 10;15(1):183. doi: 10.1186/s13023-020-01449-x. PMID: 32650830Free PMC Article
Chen CH, Lu HI, Wang YM, Chen YH, Lo CM, Huang WT, Li SH
PLoS One 2017;12(2):e0172752. Epub 2017 Feb 28 doi: 10.1371/journal.pone.0172752. PMID: 28245263Free PMC Article
Ueki H, Maeda N, Sekimizu M, Yamashita Y, Moritani S, Horibe K
J Pediatr Hematol Oncol 2014 Nov;36(8):e476-80. doi: 10.1097/MPH.0000000000000082. PMID: 24322502
Parikh SA, French CA, Costello BA, Marks RS, Dronca RS, Nerby CL, Roden AC, Peddareddigari VG, Hilton J, Shapiro GI, Molina JR
J Thorac Oncol 2013 Oct;8(10):1335-8. doi: 10.1097/JTO.0b013e3182a00f41. PMID: 24457244

Prognosis

Luo J, Sanchez M, Lee E, Hertzler H, Luong N, Mazzola E, Finstein B, Tamen R, Brisbane G, Nguyen T, Paik PK, Chaft JE, Cheng ML, Khalil H, Piha-Paul SA, Sholl LM, Nishino M, Jänne PA, DuBois SG, Hanna GJ, Shapiro GI, French CA
J Thorac Oncol 2024 May;19(5):829-838. Epub 2023 Dec 27 doi: 10.1016/j.jtho.2023.12.022. PMID: 38154515Free PMC Article
Yoshida A
Histopathology 2024 Jan;84(1):86-101. Epub 2023 Oct 24 doi: 10.1111/his.15063. PMID: 37873676
Boland JM
Mod Pathol 2022 Jan;35(Suppl 1):57-65. Epub 2021 Sep 13 doi: 10.1038/s41379-021-00914-7. PMID: 34518633
Lantuejoul S, Pissaloux D, Ferretti GR, McLeer A
Semin Diagn Pathol 2021 Sep;38(5):72-82. Epub 2021 Jun 16 doi: 10.1053/j.semdp.2021.06.005. PMID: 34176698
Fekkar A, Emprou C, Lefebvre C, Ferretti G, Stephanov O, Pissaloux D, Mc Leer A, Toffart AC, Rousseaux S, Khochbin S, Lantuejoul S, Valmary-Degano S
Lung Cancer 2021 Aug;158:55-59. Epub 2021 Jun 8 doi: 10.1016/j.lungcan.2021.06.008. PMID: 34119933

Clinical prediction guides

Yoshida A
Histopathology 2024 Jan;84(1):86-101. Epub 2023 Oct 24 doi: 10.1111/his.15063. PMID: 37873676
Lantuejoul S, Pissaloux D, Ferretti GR, McLeer A
Semin Diagn Pathol 2021 Sep;38(5):72-82. Epub 2021 Jun 16 doi: 10.1053/j.semdp.2021.06.005. PMID: 34176698
Xie XH, Wang LQ, Qin YY, Lin XQ, Xie ZH, Liu M, Zhang JX, Ouyang M, Liu J, Gu YY, Li SY, Zhou CZ
Orphanet J Rare Dis 2020 Jul 10;15(1):183. doi: 10.1186/s13023-020-01449-x. PMID: 32650830Free PMC Article
Jung M, Kim S, Lee JK, Yoon SO, Park HS, Hong SW, Park WS, Kim JE, Kim J, Keam B, Kim HJ, Kang HJ, Kim DW, Jung KC, Kim YT, Heo DS, Kim TM, Jeon YK
Oncologist 2019 Aug;24(8):e740-e748. Epub 2019 Jan 29 doi: 10.1634/theoncologist.2018-0477. PMID: 30696721Free PMC Article
Lemelle L, Pierron G, Fréneaux P, Huybrechts S, Spiegel A, Plantaz D, Julieron M, Dumoucel S, Italiano A, Millot F, Le Tourneau C, Leverger G, Chastagner P, Carton M, Orbach D
Pediatr Blood Cancer 2017 Dec;64(12) Epub 2017 Jun 23 doi: 10.1002/pbc.26693. PMID: 28643357

Recent systematic reviews

Ramesh U, Contrera KJ, Shakibai N, Su SY, Brahimaj B, Roberts D, Williams MD, Ferrarotto R, Phan J, Gunn GB, Raza S, DeMonte F, Hanna EY
Head Neck 2024 Jan;46(1):29-36. Epub 2023 Oct 18 doi: 10.1002/hed.27553. PMID: 37853958
Wang H, Weiss VL, Hoffman RD, Abel T, Ho RH, Borinstein SC, Mannion K, Bridge JA, Black J, Liang J
Head Neck Pathol 2021 Mar;15(1):236-243. Epub 2020 Feb 19 doi: 10.1007/s12105-020-01141-3. PMID: 32077054Free PMC Article

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