Background
NUT carcinoma (NC) is a rare and highly aggressive malignant tumor with unknown histologic origin and without any clinical or histomorphological features to distinguish, except genetically defined by the presence of chromosomal rearrangements involving the NUT (also known as NUTM1, Nuclear Protein in Testis) gene.1-3 In 1991, Kubonishi4 first reported a case of thymic carcinoma characterized by t(15; 19) translocation. But it was not until the discovery of formation of a BRD4-NUT fusion oncogene in 2003, resulting from the t(15;19) translocation, NC was defined.1,5
Although NC can occur in people of any age (0–81.7 years), the majority are adolescents or young adults (median age 16–22 years) and it affects males and females equally.6,7 It is also known as NMC (NUT middle carcinoma) because it typically arises from midline upper airway locations, such as head and neck and thorax. However, some cases also occur in areas other than the above organs, including: lungs,8 salivary glands,9pancreas10 and bladder1.
NC is clinically distinctly aggressive, often accompanied by extensive metastases at the time of diagnosis, and rapidly fatal.3 Because NC often occurs in the mediastinum or lungs and is usually accompanied by distant and lymph node metastases, it is often mistaken for small cell lung cancer in clinical practice, however, NC progresses more rapidly and often affects young people. Despite such a poor prognosis for NC, there are not many effective treatments and not much research on this. Herein, we report a case of pulmonary NC that achieved long-term survival after a combination of chemotherapy, radiotherapy, and immunotherapy.