Title: A Hard Nut to Crack: Case Report on Nut Midline Carcinoma- A Rare Sinonasal Malignancy
Author: Dr Aparna Rajan
DOI: https://dx.doi.org/10.18535/jmscr/v8i5.63
Abstract
NUT (Nuclear protein of testes) midline carcinoma of nasal cavity is a rare and aggressive tumour with rapid progression to adjacent sites. Diagnosis requires prompt clinical suspicion, confirmation of diagnosis and analysis of disease extend by radiology. Pathological diagnosis is by demonstration of NUT protein by Immunohistochemistry (IHC) or fusion oncogene detection by Fluorescent in situ hybridisation (FISH). Radical resection combined with chemotherapy and radiation is being tried for treatment but without much success. Newer targeted treatments are under trial. Here is a case report of a 35 year old patient with epistaxis and nasal block, diagnosed by IHC as NUT carcinoma. In spite of ongoing chemotherapy, patient expired 2 months after diagnosis which is less than the median survival period. Understanding about this rare malignancy and thinking about this rare entity among the differential diagnosis is important to prevent misdiagnosis. Successful treatment options and adequate treatment policies should be made at the earliest to prevent morbidity and mortality from this tumour.
Keywords: NUT (nuclear protein of testes), Sinonasal malignancy, poorly differentiated carcinoma.
References
- Lund VJ, Stammberger H, Nicolai P, et al. European position paper on endoscopic management of tumours of the nose, paranasal sinuses and skull base. Rhinol Suppl 2010;22: 1-143
- Den Bakker MD, Beverloo HB, van den Heuvel-Eibrink MM, Meeuwis CA, Tan ML, Johnson LA, French CA, van Leenders GJLH. NUT midline carcinoma of the parotid gland with mesenchymal differentiation. American Journal of Surgical Pathology. 2009:33(8),1253-1258
- Stelow EB, Bellizzi AM, Taneja K, Mills SE, Legallo RD, Kutok JL, et al. NUT rearrangement in undifferentiated carcinomas of the upper aerodigestive tract. Am J Surg Pathol 2008;32:828–34
- French CA. Pathogenesis of NUT midline carcinoma. Annu Rev Pathol. 2012;7:247-65.
- French CA et al BRD4-NUT fusion oncogene:a novel mechanism in aggressive carcinoma. Cancer Res. 2003;63:304-307
- Haack H, Johnson LA, Fry CJ, et al. Diagnosis of NUT midline carcinoma using a NUT-specific monoclonal antibody. Am J Surg Pathol. 2009;33(7): 984–991
- Engleson et al Midline carcinoma with BRD4-NUT fusion oncogene in a 30 year old female with response to docetaxel and radiotherapy.BMC cancer.2006;6:69
- Daniel E. Bauer, Chelsey M. Mitchell, Kelly M. Strait Clinicopathologic Features and Long-term Outcomes of NUT Midline Carcinoma Clinical cancer research 2012 18(20),5773-5779
- Bauer DE, Mitchell CM, Strait KM, Lathan CS, Stelow EB, Lure SC, et al. Clinicopathologic features andlong-term outcomes of NUT midline carcinoma. Clin Cancer Res2012;18:5773-9.
- Anastasios Stathis, Emanuele Zucca, Mohamed Bekradda. Clinical Response of Carcinomas Harboring the BRD4-NUT Oncoprotein to the Targeted Bromodomain Inhibitor OTX015/MK-8628. Cancer discovery 2016:6(5), 492-500.