NUT carcinoma (NC; formerly NUT midline carcinoma (NMC)) is a rare genetically defined, very aggressive squamous cell epithelial cancer that usually arises in the midline of the body and is characterized by a chromosomal rearrangement in the nuclear protein in testis gene (i.e. NUTM1 gene).

[2] In approximately 75% of cases, the coding sequence of NUTM1 in band 14 on the long (or "q") arm of chromosome 15 is fused to BRD4 or BRD3, which creates a chimeric gene that encodes the BRD-NUT fusion protein.

[7][8] NC when viewed microscopically, are poorly differentiated carcinomas which show abrupt transitions to islands of well-differentiated squamous epithelium.

As the mean survival under this treatment regimen is only 5–7 months,[12][7] more specific treatment options are under investigation: Specific molecular targeted therapies (including BET inhibitors and histone deacetylase inhibitors (HDACi)) may help to yield growth arrest of the neoplastic cells.

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.

Treatment must be tailored to the individual patient, with several promising new targeted molecular therapies in clinical trials.