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Nut midline carcinoma

MedGen UID:
312999
•Concept ID:
C1707291
•
Neoplastic Process
Synonyms: Carcinoma with t(15;19)(q13;p13.1) Translocation; carcinoma with t(15;19)(q13;p13.1) translocation; Midline Carcinoma of Children and Young Adults with NUT Rearrangement; Midline carcinoma of children and Young adults with NUT rearrangement; NMC; Nuclear Protein in Testis (NUT) Midline Carcinoma; Nuclear protein in testis associated carcinoma; Nuclear protein in testis carcinoma; Nuclear Protein in Testis Midline Carcinoma; nuclear protein in testis midline carcinoma; NUT (nuclear protein in testis) carcinoma; NUT (nuclear protein in testis) midline carcinoma; NUT Carcinoma; NUT carcinoma; NUT Midline Carcinoma; nut midline carcinoma; NUT Midline carcinoma; NUT midline carcinoma; NUT midline carcinoma of the head and neck
SNOMED CT: NUT (nuclear protein in testis) carcinoma (733922002); Nuclear protein in testis associated carcinoma (733922002); Nuclear protein in testis carcinoma (1187622004); NUT (nuclear protein in testis) carcinoma (1187622004); NUT (nuclear protein in testis) midline carcinoma (1187622004)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0005563
Orphanet: ORPHA443167

Definition

A rare tumour characterised by a rapidly growing mass usually arising along the midline, defined by the presence of NUTM1 rearrangements. Histopathological examination shows a poorly differentiated carcinoma, often with evidence of squamous differentiation. Patients present with unspecific signs and symptoms due to mass effect depending on the location. Extensive local invasion of adjacent structures, lymph node involvement and distant metastatic disease are often present at the time of diagnosis. Prognosis is generally poor. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVNut midline carcinoma

Professional guidelines

PubMed

Prolonged Survival of NUT Midline Carcinoma and Current Approaches to Treatment.
Kaplan HG, Subramaniam S, Vallières E, Barnett T
Oncologist 2023 Sep 7;28(9):765-770. doi: 10.1093/oncolo/oyad177. PMID: 37311046Free PMC Article
Clinical management of emerging sinonasal malignancies.
Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB
Head Neck 2020 Aug;42(8):2202-2212. Epub 2020 Mar 25 doi: 10.1002/hed.26150. PMID: 32212360
NUT Carcinoma: Clinicopathologic features, pathogenesis, and treatment.
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

Clinical features, treatment, and survival outcome of primary pulmonary NUT midline carcinoma.
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
High-Grade Sinonasal Carcinoma: Classification Through Molecular Profiling.
Guilmette J, Sadow PM
Arch Pathol Lab Med 2019 Nov;143(11):1416-1419. Epub 2019 Feb 19 doi: 10.5858/arpa.2018-0224-RS. PMID: 30779592Free PMC Article
NUT carcinoma in children and adults: A multicenter retrospective study.
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
Recently described neoplasms of the sinonasal tract.
Bishop JA
Semin Diagn Pathol 2016 Mar;33(2):62-70. Epub 2015 Dec 23 doi: 10.1053/j.semdp.2015.12.001. PMID: 26776744
NUT midline carcinoma: an imaging case series and review of literature.
Polsani A, Braithwaite KA, Alazraki AL, Abramowsky C, Shehata BM
Pediatr Radiol 2012 Feb;42(2):205-10. Epub 2011 Oct 28 doi: 10.1007/s00247-011-2272-3. PMID: 22033856

Diagnosis

Prolonged Survival of NUT Midline Carcinoma and Current Approaches to Treatment.
Kaplan HG, Subramaniam S, Vallières E, Barnett T
Oncologist 2023 Sep 7;28(9):765-770. doi: 10.1093/oncolo/oyad177. PMID: 37311046Free PMC Article
Imaging spectrum of NUT carcinomas.
Virarkar M, Saleh M, Ramani NS, Morani AC, Bhosale P
Clin Imaging 2020 Nov;67:198-206. Epub 2020 Aug 2 doi: 10.1016/j.clinimag.2020.07.025. PMID: 32866821
Clinical management of emerging sinonasal malignancies.
Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB
Head Neck 2020 Aug;42(8):2202-2212. Epub 2020 Mar 25 doi: 10.1002/hed.26150. PMID: 32212360
NUT Carcinoma: Clinicopathologic features, pathogenesis, and treatment.
French CA
Pathol Int 2018 Nov;68(11):583-595. Epub 2018 Oct 26 doi: 10.1111/pin.12727. PMID: 30362654
NUT midline carcinoma.
French CA
Cancer Genet Cytogenet 2010 Nov;203(1):16-20. doi: 10.1016/j.cancergencyto.2010.06.007. PMID: 20951314Free PMC Article

Therapy

Clinical features, treatment, and survival outcome of primary pulmonary NUT midline carcinoma.
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
NUT Midline Carcinoma of the Sublingual Gland: Clinical Presentation and Review.
Seim NB, Philips RHW, Schoenfield L, Teknos TN, Rocco JW, Agrawal A, Ozer E, Carrau RL, Kang SY, Old MO
Head Neck Pathol 2017 Dec;11(4):460-468. Epub 2017 Mar 27 doi: 10.1007/s12105-017-0809-y. PMID: 28349372Free PMC Article
Small-Molecule Targeting of BET Proteins in Cancer.
French CA
Adv Cancer Res 2016;131:21-58. Epub 2016 May 31 doi: 10.1016/bs.acr.2016.04.001. PMID: 27451123
The importance of diagnosing NUT midline carcinoma.
French CA
Head Neck Pathol 2013 Mar;7(1):11-6. Epub 2013 Mar 5 doi: 10.1007/s12105-013-0428-1. PMID: 23463074Free PMC Article
Differentiation of NUT midline carcinoma by epigenomic reprogramming.
Schwartz BE, Hofer MD, Lemieux ME, Bauer DE, Cameron MJ, West NH, Agoston ES, Reynoird N, Khochbin S, Ince TA, Christie A, Janeway KA, Vargas SO, Perez-Atayde AR, Aster JC, Sallan SE, Kung AL, Bradner JE, French CA
Cancer Res 2011 Apr 1;71(7):2686-96. Epub 2011 Mar 29 doi: 10.1158/0008-5472.CAN-10-3513. PMID: 21447744Free PMC Article

Prognosis

Imaging spectrum of NUT carcinomas.
Virarkar M, Saleh M, Ramani NS, Morani AC, Bhosale P
Clin Imaging 2020 Nov;67:198-206. Epub 2020 Aug 2 doi: 10.1016/j.clinimag.2020.07.025. PMID: 32866821
Clinical management of emerging sinonasal malignancies.
Contrera KJ, Woody NM, Rahman M, Sindwani R, Burkey BB
Head Neck 2020 Aug;42(8):2202-2212. Epub 2020 Mar 25 doi: 10.1002/hed.26150. PMID: 32212360
NUT midline carcinoma: Current concepts and future perspectives of a novel tumour entity.
Salati M, Baldessari C, Bonetti LR, Messina C, Merz V, Cerbelli B, Botticelli A
Crit Rev Oncol Hematol 2019 Dec;144:102826. Epub 2019 Nov 1 doi: 10.1016/j.critrevonc.2019.102826. PMID: 31707223
NUT Midline Carcinoma of the Sublingual Gland: Clinical Presentation and Review.
Seim NB, Philips RHW, Schoenfield L, Teknos TN, Rocco JW, Agrawal A, Ozer E, Carrau RL, Kang SY, Old MO
Head Neck Pathol 2017 Dec;11(4):460-468. Epub 2017 Mar 27 doi: 10.1007/s12105-017-0809-y. PMID: 28349372Free PMC Article
Recently described neoplasms of the sinonasal tract.
Bishop JA
Semin Diagn Pathol 2016 Mar;33(2):62-70. Epub 2015 Dec 23 doi: 10.1053/j.semdp.2015.12.001. PMID: 26776744

Clinical prediction guides

Clinical features, treatment, and survival outcome of primary pulmonary NUT midline carcinoma.
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
Tumor Mutation Burden and Checkpoint Immunotherapy Markers in NUT Midline Carcinoma.
He M, Chernock R, Zhou S, Gondim M, Dehner LP, Pfeifer JD
Appl Immunohistochem Mol Morphol 2020 Aug;28(7):495-500. doi: 10.1097/PAI.0000000000000781. PMID: 31180909
NUT carcinoma in children and adults: A multicenter retrospective study.
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
Sinonasal small round blue cell tumors: An approach to diagnosis.
Simons SA, Bridge JA, Leon ME
Semin Diagn Pathol 2016 Mar;33(2):91-103. Epub 2015 Sep 10 doi: 10.1053/j.semdp.2015.09.010. PMID: 26585346
Clinicopathologic features and long-term outcomes of NUT midline carcinoma.
Bauer DE, Mitchell CM, Strait KM, Lathan CS, Stelow EB, Lüer SC, Muhammed S, Evans AG, Sholl LM, Rosai J, Giraldi E, Oakley RP, Rodriguez-Galindo C, London WB, Sallan SE, Bradner JE, French CA
Clin Cancer Res 2012 Oct 15;18(20):5773-9. Epub 2012 Aug 15 doi: 10.1158/1078-0432.CCR-12-1153. PMID: 22896655Free PMC Article

Recent systematic reviews

Salivary Gland NUT Carcinoma with Prolonged Survival in Children: Case Illustration and Systematic Review of Literature.
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
Patterns of care and impact of prognostic factors in the outcome of NUT midline carcinoma: a systematic review and individual patient data analysis of 119 cases.
Giridhar P, Mallick S, Kashyap L, Rath GK
Eur Arch Otorhinolaryngol 2018 Mar;275(3):815-821. Epub 2018 Jan 22 doi: 10.1007/s00405-018-4882-y. PMID: 29356890

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