비부비동 영역의 NUT Midline Carcinoma 2예
Two Cases of Nuclear Protein in Testis Midline Carcinomas of Sinonasal Tract
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Nuclear protein in testis (NUT) midline carcinoma (NMC) is a rare and aggressive tumor that is genetically characterized by chromosomal rearrangement of the NUT gene. NMC predominantly involves the midline structures of the body and the sinonasal tract is considered a preferential site. While the optimal management of NMC is unclear, more than 80% of patients will die within one year of their diagnosis despite intensive treatment. We report two cases of NMC of the sinonasal tract. Histopathologic results of the punch biopsy showed undifferentiated and poorly differentiated carcinoma. NUT immunohistochemical staining results were positive. Multimodal treatments including surgery, radiotherapy, and chemotherapy were performed. We also present a literature review to compare with the present cases. In our cases, we emphasize the importance of the early diagnosis and intensive treatment of NMC. Korean J Otorhinolaryngol-Head Neck Surg 2017;60(12):673-7
Abstract
Nuclear protein in testis (NUT) midline carcinoma (NMC) is a rare and aggressive tumor that is genetically characterized by chromosomal rearrangement of the NUT gene. NMC predominantly involves the midline structures of the body and the sinonasal tract is considered a preferential site. While the optimal management of NMC is unclear, more than 80% of patients will die within one year of their diagnosis despite intensive treatment. We report two cases of NMC of the sinonasal tract. Histopathologic results of the punch biopsy showed undifferentiated and poorly differentiated carcinoma. NUT immunohistochemical staining results were positive. Multimodal treatments including surgery, radiotherapy, and chemotherapy were performed. We also present a literature review to compare with the present cases. In our cases, we emphasize the importance of the early diagnosis and intensive treatment of NMC. Korean J Otorhinolaryngol-Head Neck Surg 2017;60(12):673-7
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