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NUT carcinoma of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors: a case report

医学 肉瘤 恶性肿瘤 化疗 尤因肉瘤 鼻腔 病理 养生 原始神经外胚层肿瘤 肿瘤科 内科学 外科
作者
Kohei Arimizu,Gen Hirano,Chinatsu Makiyama,Mioko Matsuo,Toshiyuki Sasaguri,Akitaka Makiyama
出处
期刊:BMC Cancer [Springer Nature]
卷期号:18 (1) 被引量:21
标识
DOI:10.1186/s12885-018-5087-x
摘要

Nuclear protein in testis (NUT) carcinoma (NC) is a rare epithelial malignancy characterized by rearrangement of the NUT gene on chromosome 15. If NC is not suspected, it is often diagnosed as other malignancies. We present the case of NC of the nasal cavity that responded to a chemotherapy regimen for Ewing’s sarcoma family of tumors (ESFT). A 49-year-old male presented with epistaxis and pain in the left eye. The patient had a tumor in the left nasal cavity at initial visit and it was biopsied. Firstly, the man was diagnosed with ESFT based on a histopathological examination. The tumor markedly responded to standard cytotoxic chemotherapy for ESFT with distant metastasis. After the start of therapy, a chromosomal analysis revealed an atypical translocation in ESFT and additional immunostaining was positive for anti-NUT antibody. Ultimately, the patient was definitively diagnosed with NC. He received multidisciplinary therapy and symptoms were temporarily relieved. However, he died 9 months after the diagnosis of NC. When a pathologically undifferentiated tumor is evident along the midline of the body, NC must be included in the differential diagnosis, and immunohistochemical staining or genetic testing/chromosomal analysis needs to be performed.

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