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LETTER TO EDITOR  
Year : 2023  |  Volume : 66  |  Issue : 1  |  Page : 210-211
NUT carcinoma: An uncommon, yet the overlooked entity


1 Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Surgical Oncology, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

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Date of Submission27-Jul-2021
Date of Decision21-Oct-2021
Date of Acceptance22-Oct-2021
Date of Web Publication18-Jan-2023
 

How to cite this article:
Shahin M, Sahoo KK, Rebba E, Sultania M, Kar M, Sable M. NUT carcinoma: An uncommon, yet the overlooked entity. Indian J Pathol Microbiol 2023;66:210-1

How to cite this URL:
Shahin M, Sahoo KK, Rebba E, Sultania M, Kar M, Sable M. NUT carcinoma: An uncommon, yet the overlooked entity. Indian J Pathol Microbiol [serial online] 2023 [cited 2023 Feb 2];66:210-1. Available from: https://www.ijpmonline.org/text.asp?2023/66/1/210/367985




Dear Editor,

Nuclear protein in testis (NUT) carcinoma, previously referred to as NUT midline carcinoma, is an aggressive and exceedingly rare malignancy involving predominantly the upper aerodigestive tract, whereas it can affect almost any other organ of the body.[1],[2],[3] The primary pathogenesis ascribed to this neoplasm is a balanced translocation of the NUT gene present in chromosome number-15 with that of the bromodomain (BRD) family of genes, resulting in arrested squamous maturation, and increased proliferation of the immature squamous cells. As a result, this carcinoma, associated with inferior survival, is considered a form of poorly differentiated squamous cell carcinoma.[3],[4],[5] However, its histogenesis is yet unclear as other hypotheses suggest its origin from primitive neural crest-derived cells.[2]

A 19-year-old patient presented with an ulceroproliferative growth of 8.0 × 6.0 cm, involving the left lower gingivobuccal sulcus and left lip with extension into the facial skin and cervical lymphadenopathy. Histopathology of the lesion showed a diffusely infiltrating tumor with nests and lobules arrangement, along with the tumor's attachment to the mucosa's basal layer. The individual tumor cells are round to polygonal, having a high nucleocytoplasmic ratio, scant cytoplasm, hyperchromatic, and irregular nuclei with fine chromatin and conspicuous nucleoli [Figure 1]a. Many atypical mitoses, along with areas of necrosis, are noted [Figure 1]b. Few cells showing cytoplasmic clearing are also noted. Immunohistochemistry showed strong and diffuse nuclear positivity for NUT1 (speckled pattern) (1:100, clone C52B1, Cell Signalling Technology) [Figure 1]c, p40 [Figure 1]d, and retained expression of INI-1 (Ready-to-use, clone 25, path-n-situ) in tumor cells, whereas immunenegativity for HMB-45, synaptophysin, and chromogranin.
Figure 1: Microphotograph showing (a) poorly differentiated tumor predominantly at subepithelial location (H and E, 100X); (b) large areas of necrosis, conspicuous nucleoli (H and E, 400X); (c) NUT1 speckled nuclear positivity (IHC, 400X); (d) p40 nuclear positivity (IHC, 400X)

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Being an infrequent entity, this tumor has been overlooked in many scenarios. The most common differential diagnoses for this tumor are the various other poorly differentiated tumors, including round cell sarcoma, Ewing sarcoma, diffuse large cell lymphoma, desmoplastic round cell tumor, germ cell tumors, and basaloid carcinomas, etc.[6],[7] However, this tumor's differentiation from its differentials carries prime importance due to its poor prognosis and adverse patient outcome, and poor survival.[4],[8] The use of immunohistochemical marker NUT1 is essential to identify this entity with typical speckled nuclear positivity. NUT1 nuclear positivity can also be observed in germ cell tumors. However, the positivity is usually nuclear but focal and evenly distributed in the nucleus.[9] Most patients with this disease have succumbed to the disease due to widespread metastasis to multiple organs. According to a few studies, the median overall survival was found to be up to 7 months of postpresentations.[10] Though a well-established treatment guideline is not available, surgery and chemoradiation are found to be effective in some cases, provided the diagnosis is made early.[9] In conclusion, NUT midline carcinoma is one of the differentials for undifferentiated tumors of the sinonasal and oral cavity, which should not be overlooked and study of NUT1 immunohistochemistry is necessary for the confirmation.

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Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Shehata BM, Steelman CK, Abramowsky CR, Olson TA, French CA, Saxe DF, et al. NUT Midline carcinoma in a newborn with multiorgan disseminated tumor and a 2-year-old with a pancreatic/hepatic primary. Pediatr Dev Pathol 2010;13:481-5.  Back to cited text no. 1
    
2.
Harms A, Herpel E, Pfarr N, Penzel R, Heussel C-P, Herth FJ, et al. NUT carcinoma of the thorax: Case report and review of the literature. Lung Cancer 2015;90:484-91.  Back to cited text no. 2
    
3.
Mills AF, Lanfranchi M, Wein RO, Mukand-Cerro I, Pilichowska M, Cowan J, et al. NUT midline carcinoma: A case report with a novel translocation and review of the literature. Head Neck Pathol 2014;8:182-6.  Back to cited text no. 3
    
4.
Seim NB, Philips RH, Schoenfield L, Teknos TN, Rocco JW, Agrawal A, et al. NUT midline carcinoma of the sublingual gland: Clinical presentation and review. Head Neck Pathol 2017;11:460-8.  Back to cited text no. 4
    
5.
Vakani P, Maheshwari J, Maheshwari M, Shah B. Sinonasal NUT midline carcinoma: A new histological entity. Indian J Pathol Microbiol 2020;63:103-5.  Back to cited text no. 5
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6.
Suzuki S, Kurabe N, Minato H, Ohkubo A, Ohnishi I, Tanioka F, et al. A rare Japanese case with a NUT midline carcinoma in the nasal cavity: A case report with immunohistochemical and genetic analyses. Pathol-Res Pract 2014;210:383-8.  Back to cited text no. 6
    
7.
Edgar M, Caruso AM, Kim E, Foss RD. NUT midline carcinoma of the nasal cavity. Head Neck Pathol 2017;11:389-92.  Back to cited text no. 7
    
8.
Joel S, Weschenfelder F, Schleussner E, Hofmann GO, Weschenfelder W. NUT midline carcinoma in a young pregnant female: A case report. World J Surg Oncol 2020;18:290.  Back to cited text no. 8
    
9.
Elkhatib SK, Neilsen BK, Sleightholm RL, Baine MJ, Zhen W. A 47-year-old woman with nuclear protein in testis midline carcinoma masquerading as a sinus infection: A case report and review of the literature. J Med Case Reports 2019;13:57.  Back to cited text no. 9
    
10.
Iacobelli JF, Charles AK, Crook M, Stewart CJ. NUT protein immunoreactivity in ovarian germ cell tumours. Pathology 2015;47:118-22.  Back to cited text no. 10
    

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Correspondence Address:
Mukund Sable
Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Bhubaneswar, Odisha
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/IJPM.IJPM_772_21

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