纵隔：NUT Carcinoma ？讨论病例【91360.软组织650】

刘伟

一公里生命
病例(形态)分享第422例
48y old F with hemorrhagic pleurisy. A biopsy of a pleural lesion was performed. What is your Dx



神奇的东方树叶
BRG1？


邓天朝
尤文样
NUT重排


伊人一方
间皮，癌，大间变等


神奇的东方树叶
细胞单一圆形细胞，泡状核，胞质似乎透亮，坏死恒定出现，核分裂象易见，无H-W菊形团，无PP，但是这种扩张的厚壁血管令人困惑
我选择恶性血管球瘤


一公里生命
NUT Carcinoma


Poor prognosis(surv <1yr)


Frequent midline location


Sheets of poorly differentiated epithelioid cells


Necrosis and mitosis+++


Abrupt squamous diff in some


EMA/CK+, CD34+ (50%), p40/p63 (focal), NUT+ (speckled)


BRD4-NUT fusion (most)



一公里生命
DDx: Poorly diff carc, rhabdoid tumors, SMARCA4-Deficient Neoplasms, melanoma, lymphoma.


Chemoresistant. Some response to BET inhibitors but high toxicity.


CIC-NUTM1 fusions can be seen in some pediatric sarcomas of the CNS
注：病例来自网络，版权归原作者仅供学习