NUT midline carcinomas are rare epithelial malignancies. In 2016, only 48 patients with this tumor involving the head and neck were described, and ours is the second case report from Japan. These tumors are characterized by the presence of thet (15 ; 19) chromosomal translocation, difficulty in pathological diagnosis, young-onset, aggressive nature of the tumor and resistance to standard cancer therapy. We report the case of a 49 -year- old man who was diagnosed as having sinonasal NUT midline carcinoma. He had bone metastasis, and his tumor grew rapidly within a week. The initial diagnosis was Ewing sarcoma, however, the final diagnosis was NUT midline carcinoma with a t (15 ; 19) chromosomal translocation. At least once, a sarcoma-based chemotherapy regimen was effective in reducing the size of the tumor, however, the tumor regrew and became resistant to cisplatin-based chemoradiotherapy and other chemotherapies. The patient died of his disease less than 9 months after the initial diagnosis. For early diagnosis and treatment of NUT midline carcinoma, it is important to have a high index of suspicion for this disease.
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