Scientific Letter
NUT Midline Carcinoma of the Lung, A Rare Form of Lung Cancer
Carcinoma NUT pulmonar, una forma poco frecuente de cáncer de pulmón
Cristina Benito Bernáldez
, Concepción Romero Muñoz, Virginia Almadana Pacheco
Corresponding author
Servicio de Neumología, Hospital Universitario Virgen Macarena, Sevilla, Spain
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class="elsevierStyleSimplePara elsevierViewall">(A) Axial image of chest CT (pulmonary parenchymal window) showing linear thickening of the subpleural lung interstitium (arrows). 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They often affect children and young adults. They may occur in different organs, but they are characteristically located in the midline of the head and neck.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Very few cases have been published that describe this entity in the lungs and mediastinum.</p><p id="par0010" class="elsevierStylePara elsevierViewall">We report the case of a 23-year-old man, smoker of about 10 cigarettes/day, who presented in our hospital with a 2-week history of pain in the right hemithorax radiating to the shoulder, and asthenia. Computed tomography (CT) showed increased density without air bronchogram occupying the posterior and apical segments of the right upper lobe (RUL) with amputation of the corresponding segmentary bronchi, paratracheal mediastinal lymphadenopathies, and significant right pleural effusion (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). Given these findings, a positron emission tomography (PET) was performed, revealing a metabolically positive mass in the RUL, with extensive pleural involvement in the same side, mediastinal lymph node involvement, and bilateral supra/infraclavicular lytic bone lesions and metastasis. Fiberoptic bronchoscopy revealed complete stenosis of the posterior and apical segments of the RUL bronchus due to thickened, hypervascularized mucosa, possibly of a neoformative origin. Diagnostic confirmation was obtained from a pleural biopsy obtained by thoracoscopy. The pathology report described a neoplasm formed of nests of undifferentiated cells, and, along with this cell population, abrupt areas of squamous differentiation. The undifferentiated tumor cells showed positive immunoreaction to NUT antibody and pancytokeratin (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">Given the definitive diagnosis of NUT carcinoma, the patient received 2 cycles of palliative chemotherapy with cisplatin and etoposide, and then stared BET inhibitor therapy in a clinical trial setting. The oncological disease did not respond to treatment, and the pulmonary mass increased in size, with compression of the superior vena cava and pericardial effusion. The patient died 3 months after the initial diagnosis.</p><p id="par0020" class="elsevierStylePara elsevierViewall">NUT carcinomas are poorly differentiated and genetically defined by the presence of a genetic rearrangement of the NUT gene, consisting of a chromosomal translocation between this gene (NUTM1) located in the chromosome 15q14 and other genes (BRD4 in chromosome 19p13.1 [70%], BRD3 in chromosome 9q34.2 [6%] or an unknown gene [24%]).<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">2</span></a> The incidence is unknown, but this tumor is considered rare. It was originally thought to be a disease of children and young adults, but subsequent publications have shown that NUT carcinoma can equally affect individuals of any age and both sexes.</p><p id="par0025" class="elsevierStylePara elsevierViewall">The tumor generally occurs in the midline structures, usually associated with the upper gastrointestinal tract, but it has been identified in other sites. According to reports in the literature, only 38 cases of primary intrathoracic NUT are known, of which 19 were of pulmonary origin.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">In histopathological terms, it is very similar to squamous carcinoma, and therefore may be underdiagnosed. If NUT is suspected, diagnostic confirmation is obtained with immunohistochemical techniques, as in our patient, or by the detection of chromosomal rearrangement of the specific NUT variants.<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a></p><p id="par0035" class="elsevierStylePara elsevierViewall">To date, no specific treatment exists for NUT carcinoma. The rapid growth and aggressivity of this tumor rules out surgical treatment, and it does not appear to respond to most chemotherapeutic regimens and/or radiation therapy, although the best option appears to be a combination of gemcitabine, docetaxel and cisplatin. Some promising therapies, such as vorinostat and BET inhibitors, are currently under study and perhaps these will provide an effective treatment in the future.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> However, the current prognosis for this entity is death after a mean period of survival of around 7 months,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">5</span></a> as occurred in our case. To conclude, NUT carcinoma is extremely rare and very aggressive, so early clinical suspicion of this entity of utmost importance to avoid delays in establishing the most specific treatment possible in the shortest time.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Benito Bernáldez C, Romero Muñoz C, Almadana Pacheco V. Carcinoma NUT pulmonar, una forma poco frecuente de cáncer de pulmón. Arch Bronconeumol. 2016;52:619–621.</p>" ] ] "multimedia" => array:1 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Fig. 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1350 "Ancho" => 1800 "Tamanyo" => 430772 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Computed tomography images showing the pulmonary lesion in the right upper lobe, and images from the pathological examination showing undifferentiated tumor cells (A) and positive immunoreaction to NUT antibody (B).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:5 [ 0 => array:3 [ "identificador" => "bib0030" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "NUT midline carcinoma a case report with a novel translocation and review of the literature" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "A.F. 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Lüer" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1158/1078-0432.CCR-12-1153" "Revista" => array:6 [ "tituloSerie" => "Clin Cancer Res" "fecha" => "2012" "volumen" => "18" "paginaInicial" => "5773" "paginaFinal" => "5779" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/22896655" "web" => "Medline" ] ] ] ] ] ] ] ] ] ] ] ] ] "idiomaDefecto" => "en" "url" => "/15792129/0000005200000012/v1_201611260059/S1579212916302233/v1_201611260059/en/main.assets" "Apartado" => array:4 [ "identificador" => "49861" "tipo" => "SECCION" "en" => array:2 [ "titulo" => "Scientific letters" "idiomaDefecto" => true ] "idiomaDefecto" => "en" ] "PDF" => "https://static.elsevier.es/multimedia/15792129/0000005200000012/v1_201611260059/S1579212916302233/v1_201611260059/en/main.pdf?idApp=UINPBA00003Z&text.app=https://archbronconeumol.org/" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S1579212916302233?idApp=UINPBA00003Z" ]
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| 2024 April | 63 | 29 | 92 |
| 2024 March | 58 | 21 | 79 |
| 2024 February | 46 | 27 | 73 |
| 2023 March | 11 | 6 | 17 |
| 2023 February | 72 | 23 | 95 |
| 2023 January | 56 | 24 | 80 |
| 2022 December | 68 | 37 | 105 |
| 2022 November | 72 | 26 | 98 |
| 2022 October | 99 | 35 | 134 |
| 2022 September | 62 | 36 | 98 |
| 2022 August | 67 | 40 | 107 |
| 2022 July | 56 | 39 | 95 |
| 2022 June | 75 | 44 | 119 |
| 2022 May | 73 | 41 | 114 |
| 2022 April | 104 | 34 | 138 |
| 2022 March | 103 | 43 | 146 |
| 2022 February | 112 | 36 | 148 |
| 2022 January | 108 | 42 | 150 |
| 2021 December | 89 | 43 | 132 |
| 2021 November | 131 | 50 | 181 |
| 2021 October | 125 | 63 | 188 |
| 2021 September | 92 | 46 | 138 |
| 2021 August | 104 | 68 | 172 |
| 2021 July | 142 | 67 | 209 |
| 2021 June | 236 | 47 | 283 |
| 2021 May | 371 | 51 | 422 |
| 2021 April | 484 | 169 | 653 |
| 2021 March | 230 | 50 | 280 |
| 2021 February | 152 | 33 | 185 |
| 2021 January | 162 | 41 | 203 |
| 2020 December | 165 | 22 | 187 |
| 2020 November | 210 | 24 | 234 |
| 2020 October | 127 | 22 | 149 |
| 2020 September | 170 | 26 | 196 |
| 2020 August | 224 | 41 | 265 |
| 2020 July | 154 | 28 | 182 |
| 2020 June | 183 | 18 | 201 |
| 2020 May | 192 | 10 | 202 |
| 2020 April | 183 | 27 | 210 |
| 2020 March | 220 | 24 | 244 |
| 2020 February | 389 | 41 | 430 |
| 2020 January | 251 | 33 | 284 |
| 2019 December | 222 | 24 | 246 |
| 2019 November | 257 | 29 | 286 |
| 2019 October | 167 | 24 | 191 |
| 2019 September | 172 | 17 | 189 |
| 2019 August | 133 | 18 | 151 |
| 2019 July | 229 | 17 | 246 |
| 2019 June | 217 | 20 | 237 |
| 2019 May | 286 | 41 | 327 |
| 2019 April | 206 | 34 | 240 |
| 2019 March | 170 | 34 | 204 |
| 2019 February | 122 | 33 | 155 |
| 2019 January | 115 | 22 | 137 |
| 2018 December | 94 | 19 | 113 |
| 2018 November | 130 | 26 | 156 |
| 2018 October | 125 | 35 | 160 |
| 2018 September | 68 | 15 | 83 |
| 2018 May | 23 | 1 | 24 |
| 2018 April | 50 | 8 | 58 |
| 2018 March | 42 | 4 | 46 |
| 2018 February | 78 | 7 | 85 |
| 2018 January | 113 | 22 | 135 |
| 2017 December | 79 | 8 | 87 |
| 2017 November | 46 | 12 | 58 |
| 2017 October | 28 | 5 | 33 |
| 2017 September | 27 | 14 | 41 |
| 2017 August | 39 | 19 | 58 |
| 2017 July | 37 | 13 | 50 |
| 2017 June | 45 | 24 | 69 |
| 2017 May | 29 | 14 | 43 |
| 2017 April | 37 | 15 | 52 |
| 2017 March | 26 | 17 | 43 |
| 2016 December | 0 | 1 | 1 |
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