37:
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rhabdomyosarcoma, and melanoma. When NUT midline carcinoma is seen in the head and neck, the squamous lining of the cavities may be entrapped by the neoplastic cells, and so it is important to document the carcinoma cells in the rest of the tumor by a variety of stains (including cytokeratin or p63). One of the most helpful and characteristic findings is the focal abrupt squamous differentiation, where stratification and gradual differentiation are absent, resembling a
Hassall corpuscle of the thymus.
253:(HDACi)) may help to yield growth arrest of the neoplastic cells. Two genetically engineered mouse models (GEMMs) of NC have been reported by the French lab (2023 ) and Gu lab (2024 ). These models demonstrate that BRD4-NUT alone drives malignant transformation and spread of NUT carcinoma and will help enable the discovery and development of novel, targeted therapies for NC.
235:
is quite wide, but it is important to consider this tumor type when seeing a poorly differentiated tumor that shows abrupt areas of keratinization. Other tumors included in the differential diagnosis are sinonasal undifferentiated carcinomas, Ewing sarcoma/Primitive neuroectodermal tumor, leukemia,
261:
NUT carcinoma is very resistant to standard chemotherapy treatments. The tumor may initially respond to therapy, and then rapid recurrence is experienced. A multimodality approach to treatment is advocated, especially since most patients present with advanced disease. Treatment must be tailored to
184:
NC when viewed microscopically, are poorly differentiated carcinomas which show abrupt transitions to islands of well-differentiated squamous epithelium. This tumor pattern is not specific or unique to NUT carcinoma, but this pattern is most suggestive of the diagnosis. The neoplastic cells will
828:
Durall, R Taylor; Huang, Julianna; Wojenski, Luke; Huang, Yeying; Gokhale, Prafulla C; Leeper, Brittaney A; Nash, Joshua O; Ballester, Pedro L; Davidson, Scott; Shlien, Adam; Sotirakis, Emmanuel; Bertaux, Fabien; Dubus, Vincent; Luo, Jia; Wu, Catherine J; Keskin, Derin B; Eagen, Kyle P; Shapiro,
604:
Bauer, Daniel E; Mitchell, Chelsey M; Strait, Kelly M; Lathan, Christopher S; Stelow, Edward B; Lüer, Sonja C; Muhammed, Somala; Evans, Andrew G; Sholl, Lynette M; Rosai, Juan; Giraldi, Eugenia; Oakley, Richard P; Rodriguez-Galindo, Carlos; London, Wendy B; Sallan, Stephen E; Bradner, James E;
244:
Currently, the standard treatment approach is a multimodal approach involving surgery as well as radio-chemotherapy. As the mean survival under this treatment regimen is only 5–7 months, more specific treatment options are under investigation: Specific molecular targeted therapies (including
164:) may be seen, site specific symptoms are also present. If the tumor involves the head and neck region (in about 35%), then pain, a mass, obstructive symptoms, among others, may be experienced. NUT carcinomas are not specific to any tissue type or organ. Common sites include the
155:
The number of cases per year is likely grossly underestimated. One study suggests the incidence rate is likely around 1,000 cases per year in the United States. The symptoms are similar to other forms of cancer and dependent on the stage. While generalized symptoms
962:
770:
Bauer, Daniel E.; Mitchell, Chelsey M.; Strait, Kelly M.; Lathan, Christopher S.; Stelow, Edward B.; Lüer, Sonja C.; Muhammed, Somala; Evans, Andrew G.; Sholl, Lynette M.; Rosai, Juan; Giraldi, Eugenia (2012-08-15).
870:
Zheng, Dejin; Elnegiry, Ahmed A; Luo, Chenxiang; Bendahou, Mohammed Amine; Xie, Liangqi; Bell, Diana; Takahashi, Yoko; Hanna, Ehab; Mias, George I; Tsoi, Mayra F; Gu, Bin (May 2024).
317:
551:"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"
381:
395:
Stevens, Todd M; Morlote, Diana; Xiu, Joanne; Swensen, Jeffrey; Weber-Brandwein, Margaret; Miettinen, Markku M; Gatalica, Zoran; Bridge, Julia A (Feb 2019).
223:(t(15;19)(q13;p13.1)). Therefore, staining with the NUT antibody confirms the diagnosis (although only available in a limited number of laboratories).
262:
the individual patient, with several promising new targeted molecular therapies in clinical trials. Overall, there is a mean survival of 6–9 months.
606:
357:
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645:
330:
397:"NUTM1-rearranged neoplasia: a multi-institution experience yields novel fusion partners and expands the histologic spectrum"
97:
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352:. William D. Travis, Elisabeth Brambilla, Allen Burke, Alexander Marx, Andrew G. Nicholson (4th ed.). Lyon.
212:
144:
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83:
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143:
The name NUT carcinoma was introduced as the carcinoma does not only occur in the body midline; therefore,
36:
147:
also changed the name in 2015 in the WHO Classification of
Tumours of the Lung, Pleura, Thymus and Heart.
1014:
136:
fusion protein. The remaining cases, the fusion of NUTM1 is to an unknown partner gene, usually called
201:
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of a NUT carcinoma with the characteristic well-differentiated islands of squamous epithelium.
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Giridhar, Prashanth; Mallick, Supriya; Kashyap, Lakhan; Rath, Goura Kishor (2018-01-22).
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French, CA. (Jun 2010). "Demystified molecular pathology of NUT midline carcinomas".
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176:. The median age at diagnosis is 16–23 years, but older patients may be affected.
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367:
831:"The BRD4-NUT Fusion Alone Drives Malignant Transformation of NUT Carcinoma"
773:"Clinicopathologic Features and Long-term Outcomes of NUT Midline Carcinoma"
607:"Clinicopathologic Features and Long-term Outcomes of NUT Midline Carcinoma"
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109:
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that usually arises in the midline of the body and is characterized by a
954:
663:"NUT Carcinoma: Clinicopathologic features, pathogenesis, and treatment"
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662:
966:
89:
349:
WHO Classification of
Tumours of the Lung, Pleura, Thymus and Heart
649:
334:
216:
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122:
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However, the defining feature of NCs is rearrangement of the
104:
gene). In approximately 75% of cases, the coding sequence of
872:"Brd4::Nutm1 fusion gene initiates NUT carcinoma in vivo"
930:"International NUT Midline Carcinoma Registry - Doctors"
944:
723:"The importance of diagnosing NUT midline carcinoma"
346:
International Agency for
Research on Cancer (2015).
978:
948:
55:
26:
21:
82:)) is a rare genetically defined, very aggressive
132:, which creates a chimeric gene that encodes the
829:Geoffrey I; French, Christopher A (Dec 2023).
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8:
316:: CS1 maint: numeric names: authors list (
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555:European Archives of Oto-Rhino-Laryngology
380:: CS1 maint: location missing publisher (
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185:show a positive reaction with various
661:French, Christopher A. (2018-10-26).
7:
544:
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291:RESERVED, INSERM US14-- ALL RIGHTS.
646:Online Mendelian Inheritance in Man
467:10.1016/j.cancergencyto.2010.06.007
331:Online Mendelian Inheritance in Man
605:French, Christopher A (Oct 2012).
14:
293:"Orphanet: NUT midline carcinoma"
251:histone deacetylase inhibitors
98:nuclear protein in testis gene
1:
847:10.1158/0008-5472.CAN-23-2545
789:10.1158/1078-0432.ccr-12-1153
623:10.1158/1078-0432.CCR-12-1153
30:NUT midline carcinoma (NMC)
1031:
739:10.1007/s12105-013-0428-1
567:10.1007/s00405-018-4882-y
413:10.1038/s41379-019-0206-z
94:chromosomal rearrangement
43:
34:
777:Clinical Cancer Research
611:Clinical Cancer Research
449:French, CA. (Nov 2010).
727:Head and Neck Pathology
667:Pathology International
517:10.1136/jcp.2007.052902
451:"NUT midline carcinoma"
211:gene. Most common is a
889:10.26508/lsa.202402602
721:French, C. A. (2013).
455:Cancer Genet Cytogenet
233:differential diagnosis
227:Differential diagnosis
918:10.1056/NEJMra1112635
876:Life Science Alliance
76:NUT midline carcinoma
202:immunohistochemistry
979:External resources
151:Signs and symptoms
22:NUT carcinoma (NC)
1002:
1001:
841:(23): 3846–3860.
783:(20): 5773–5779.
680:10.1111/pin.12727
617:(20): 5773–5779.
359:978-92-832-2436-5
114:long (or "q") arm
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16:Medical condition
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84:squamous cell
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68:NUT carcinoma
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49:H&E stain
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1015:Rare cancers
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511:(6): 492–6.
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300:. Retrieved
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187:cytokeratins
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120:is fused to
105:
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79:
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71:
67:
66:
733:(1): 11–6.
302:18 November
174:mediastinum
158:weight loss
140:-variant.
74:; formerly
27:Other names
278:References
112:14 on the
87:epithelial
45:Micrograph
797:1078-0432
689:1320-5463
575:0937-4477
376:cite book
368:898924651
257:Prognosis
240:Treatment
219:gene and
180:Diagnosis
57:Specialty
1009:Category
988:Orphanet
899:11082452
856:10690098
815:22896655
757:23463074
705:53104234
697:30362654
648:(OMIM):
583:29356890
525:18552174
485:20951314
431:30723300
333:(OMIM):
312:cite web
266:See also
221:NUT gene
61:Oncology
970:: C80.9
806:3473162
748:3597165
632:3473162
591:3379707
533:2200842
476:3000636
422:8194366
162:fatigue
134:BRD-NUT
96:in the
993:443167
896:
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650:608749
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429:
419:
366:
356:
335:608963
197:, and
100:(i.e.
90:cancer
882:(7).
701:S2CID
587:S2CID
529:S2CID
106:NUTM1
102:NUTM1
811:PMID
793:ISSN
753:PMID
693:PMID
685:ISSN
579:PMID
571:ISSN
521:PMID
481:PMID
427:PMID
382:link
364:OCLC
354:ISBN
318:link
304:2019
249:and
231:The
217:BRD4
199:CD34
172:and
170:neck
166:head
160:and
129:BRD3
123:BRD4
110:band
963:ICD
914:doi
894:PMC
884:doi
851:PMC
843:doi
801:PMC
785:doi
743:PMC
735:doi
675:doi
627:PMC
619:doi
563:doi
559:275
513:doi
471:PMC
463:doi
459:203
417:PMC
409:doi
209:NUT
195:CEA
191:p63
145:WHO
138:NUT
126:or
116:of
108:in
80:NMC
1011::
991::
967:10
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878:.
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Text is available under the Creative Commons Attribution-ShareAlike License. Additional terms may apply.
