NUT Midline Carcinoma: Case Report and Review of the Literature

被引:21
|
作者
Hsieh, Min-Shu [2 ]
French, Christopher A. [3 ]
Liang, Cher-Wei [4 ]
Hsiao, Cheng-Hsiang [1 ]
机构
[1] Natl Taiwan Univ Hosp, Dept Pathol, Taipei 100, Taiwan
[2] Natl Taiwan Univ Hosp, Yun Lin Branch, Yunlin, Taiwan
[3] Brigham & Womens Hosp, Boston, MA 02115 USA
[4] Cathay Gen Hosp, Taipei, Taiwan
关键词
NUT midline carcinoma; t(15; 19) translocation; BRD4-NUT; BROMODOMAIN PROTEIN BRD4; AGGRESSIVE CARCINOMA; FUSION ONCOGENE; P-TEFB; TRANSLOCATION; REARRANGEMENT; TRANSCRIPTION; PROGRESSION; T(15/19); TRACT;
D O I
10.1177/1066896909353600
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
NUT midline carcinoma (NMC) is a recently described, undifferentiated carcinoma with specific NUT gene rearrangement, which often involves midline organs such as the nasal cavity, paranasal sinuses, mediastinum, or intrathoracic organs. It was previously considered a disease of children or young adults, but middle-aged or elderly patients have subsequently been seen. Here, the authors report the case of a 54-year-old woman who presented with a left-nasal-cavity mass and diplopia. The tumor enlarged rapidly and extended to the left orbital cavity and brain base despite chemotherapy and radiotherapy. Pathological examination of the resected tumor showed an undifferentiated carcinoma with occasional abrupt keratinizing squamous differentiation. Immunohistochemical analysis with an antibody to NUT revealed that most of the tumor cells were positive. BRD4-NUT gene fusion was demonstrated by fluorescence in situ hybridization, confirming the diagnosis of NMC. This case emphasizes the importance of considering NMC in the differential diagnosis in older adults.
引用
收藏
页码:808 / 812
页数:5
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