International guidelines on the diagnosis and treatment of NUT carcinoma

Yu Zhang; Qi Zhang; Yue Hao; Jia Luo; Yingshi Piao; Wenxian Wang; Zhengbo Song; Ziming Li; Luka Brcic; Aijun Liu; Jinpu Yu; Yasuhiro Tsutani; Wenzhao Zhong; Wenfeng Fang; Zhijie Wang; Shengxiang Ren; Athanasios G. Papavassiliou; Yongchang Zhang; Jingjing Liu; Shirong Zhang; Xiuyu Cai; Ayten Kayi Cangir; Anwen Liu; Wen Li; Filippo Lococo; Ping Zhan; Hongbing Liu; Tangfeng Lv; Liyun Miao; Lingfeng Min; Helmut Popper; Yu Chen; Jingping Yuan; Feng Wang; Zhansheng Jiang; Gen Lin; Long Huang; Xingxiang Pu; Rongbo Lin; Kalevi Kairemo; Weifeng Liu; Chuangzhou Rao; Dongqing Lv; Zongyang Yu; Ashrafian Leanne; Xiaoyan Li; Chuanhao Tang; Hifzur R. Siddique; Chengzhi Zhou; Junping Zhang; Junli Xue; Vishal Shelat; Hui Guo; Qian Chu; Rui Meng; Fatemeh Ardeshir; Jingxun Wu; Rui Zhang; Jin Zhou; Robert A. Kratzke; Zhengfei Zhu; Yongheng Li; Hong Qiu; Fan Xia; Fiorella Calabrese; Yang Xia; Alessandro Wasum Mariani; Yuanyuan Lu; Xiaofeng Chen; Mark A. Klein; Rui Ge; Enyong Dai; Axel H. Schönthal; Yu Han; Zhenying Guo; Jian Zhang; Yinghua Ji; Xianbin Liang; Hongmei Zhang; Xuelei Ma; Marco Chiappetta; Xuewen Liu; Francoise Galateau Salle; Yu Yao; Malgorzata Szolkowska; Weiwei Pan; Fei Pang; Fan Wu; Stefan B. Watzka; Liping Wang; Youcai Zhu; Li Lin; Aparna Sharma; Jianfei Tu; Xinqing Lin; Jing Cai; Ling Xu; Jisheng Li; Xiaodong Jiao; Kainan Li; Marjorie G. Zauderer; Jia Wei; Huijing Feng; Lin Wang; Yingying Du; Wang Yao; Elizabeth Dudnik; Xuefei Shi; Xiaomin Niu; Dongmei Yuan; Yanwen Yao; Jianhui Huang; Yue Feng; Yinbin Zhang; Binbin Song; Wenfeng Li; Jianfei Fu; Marina K. Baine; Pingli Sun; Hong Wang; Mingxiang Ye; Dong Wang; Zhaofeng Wang; Jing Wu; Yunyun Yang; Yuan Fang; Zhen Wang; Bin Wan; Donglai Lv; Huafei Chen; Shengjie Yang; Jing Kang; Jiatao Zhang; Chao Zhang; Lin Shi; Yina Wang; Mohamed Emam Sobeih; Bihui Li; Bin Lian; Lili Mao; Zhang Zhang; Ke Wang; Zhongwu Li; Zhefeng Liu; Nong Yang; Lin Wu; Xiaobing Chen; Gu Jin; Miao Li; Guansong Wang; Thomas U. Marron; Jiandong Wang; Sanjay Popat; Meiyu Fang; Yong Fang; Daniel Mansilla; Yuan Li; Xiaojia Wang; Jing Chen; Yiping Zhang; Xixu Zhu; Yi Shen; Shenglin Ma; Aaron S. Mansfield; Biyun Wang; Lu Si; Anja C. Roden; Bjørn H. Grønberg; Yong Song; Geoffrey I. Shapiro; Christopher A. French; Yuanzhi Lu; Qian Wang; Chunwei Xu

doi:10.1016/j.xinn.2025.101068

International guidelines on the diagnosis and treatment of NUT carcinoma

Yu Zhang
, Qi Zhang
, Yue Hao
, Jia Luo
, Yingshi Piao
, Wenxian Wang
, Zhengbo Song
, Ziming Li
, Luka Brcic
, Aijun Liu
, Jinpu Yu
, Yasuhiro Tsutani
, Wenzhao Zhong
, Wenfeng Fang
, Zhijie Wang
, Shengxiang Ren
, Athanasios G. Papavassiliou
, Yongchang Zhang
, Jingjing Liu
, Shirong Zhang

Xiuyu Cai, Ayten Kayi Cangir, Anwen Liu, Wen Li, Filippo Lococo, Ping Zhan, Hongbing Liu, Tangfeng Lv, Liyun Miao, Lingfeng Min, Helmut Popper, Yu Chen, Jingping Yuan, Feng Wang, Zhansheng Jiang, Gen Lin, Long Huang, Xingxiang Pu, Rongbo Lin, Kalevi Kairemo, Weifeng Liu, Chuangzhou Rao, Dongqing Lv, Zongyang Yu, Ashrafian Leanne, Xiaoyan Li, Chuanhao Tang, Hifzur R. Siddique, Chengzhi Zhou, Junping Zhang, Junli Xue, Vishal Shelat, Hui Guo, Qian Chu, Rui Meng, Fatemeh Ardeshir, Jingxun Wu, Rui Zhang, Jin Zhou, Robert A. Kratzke, Zhengfei Zhu, Yongheng Li, Hong Qiu, Fan Xia, Fiorella Calabrese, Yang Xia, Alessandro Wasum Mariani, Yuanyuan Lu, Xiaofeng Chen, Mark A. Klein, Rui Ge, Enyong Dai, Axel H. Schönthal, Yu Han, Zhenying Guo, Jian Zhang, Yinghua Ji, Xianbin Liang, Hongmei Zhang, Xuelei Ma, Marco Chiappetta, Xuewen Liu, Francoise Galateau Salle, Yu Yao, Malgorzata Szolkowska, Weiwei Pan, Fei Pang, Fan Wu, Stefan B. Watzka, Liping Wang, Youcai Zhu, Li Lin, Aparna Sharma, Jianfei Tu, Xinqing Lin, Jing Cai, Ling Xu, Jisheng Li, Xiaodong Jiao, Kainan Li, Marjorie G. Zauderer, Jia Wei, Huijing Feng, Lin Wang, Yingying Du, Wang Yao, Elizabeth Dudnik, Xuefei Shi, Xiaomin Niu, Dongmei Yuan, Yanwen Yao, Jianhui Huang, Yue Feng, Yinbin Zhang, Binbin Song, Wenfeng Li, Jianfei Fu, Marina K. Baine, Pingli Sun, Hong Wang, Mingxiang Ye, Dong Wang, Zhaofeng Wang, Jing Wu, Yunyun Yang, Yuan Fang, Zhen Wang, Bin Wan, Donglai Lv, Huafei Chen, Shengjie Yang, Jing Kang, Jiatao Zhang, Chao Zhang, Lin Shi, Yina Wang, Mohamed Emam Sobeih, Bihui Li, Bin Lian, Lili Mao, Zhang Zhang, Ke Wang, Zhongwu Li, Zhefeng Liu, Nong Yang, Lin Wu, Xiaobing Chen, Gu Jin, Miao Li, Guansong Wang, Thomas U. Marron, Jiandong Wang, Sanjay Popat, Meiyu Fang, Yong Fang, Daniel Mansilla, Yuan Li, Xiaojia Wang, Jing Chen, Yiping Zhang, Xixu Zhu, Yi Shen, Shenglin Ma, Aaron S. Mansfield, Biyun Wang, Lu Si, Anja C. Roden, Bjørn H. Grønberg, Yong Song, Geoffrey I. Shapiro, Christopher A. French, Yuanzhi Lu, Qian Wang, Chunwei Xu

Health Science Center

Research output: Contribution to journal › Review article › peer-review

4 Scopus citations

Abstract

Nuclear protein in testis (NUT) carcinoma (NC) represents a rare, clinically aggressive cancer defined by pathognomonic NUT Midline Carcinoma Family Member 1 (NUTM1) gene fusions, with bromodomain and extraterminal domain (BET) protein 4 (BRD4)-NUTM1 being the predominant oncogenic driver. Since its description in 1991, gradual advances have clarified the pathologic mechanisms of NC and its diagnostic methods; however, NC treatment remains a significant challenge. Moreover, diagnostic and treatment approaches for this cancer require further validation and standardization. These guidelines were developed by the Chinese Alliance of Research for NC (ChARN) based on current evidence in the literature and incorporate consensus-based input from multiple international experts. They provide comprehensive guidance on NC diagnosis and treatment, covering epidemiology, pathogenesis, diagnostic methods, therapeutic strategies, BET-inhibitor toxicity, palliative care, and prognostic assessment during follow-up. They also emphasize the importance of multidisciplinary team collaboration in NC treatment and recommend prioritizing enrollment in prospective clinical trials for patients. Current mainstays of treatment include surgical resection, radiotherapy, and medical treatment (chemotherapy, targeted therapy, and immunotherapy), although no standard treatment protocol exists. Future research directions include improving diagnostic efficiency, exploring new therapeutic strategies (such as highly selective BET inhibitors, BET-inhibitor combinations, and PROTAC technologies), and recommending basket trials as a research approach for patients with NUTM1 gene fusions.

Original language	English
Article number	101068
Journal	Innovation
DOIs	https://doi.org/10.1016/j.xinn.2025.101068
State	Accepted/In press - 2025

Keywords

diagnosis
guideline
NUT carcinoma
treatment

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1016/j.xinn.2025.101068

Cite this

Zhang, Y., Zhang, Q., Hao, Y., Luo, J., Piao, Y., Wang, W., Song, Z., Li, Z., Brcic, L., Liu, A., Yu, J., Tsutani, Y., Zhong, W., Fang, W., Wang, Z., Ren, S., Papavassiliou, A. G., Zhang, Y., Liu, J., ... Xu, C. (Accepted/In press). International guidelines on the diagnosis and treatment of NUT carcinoma. Innovation, Article 101068. https://doi.org/10.1016/j.xinn.2025.101068

@article{40855537da74480da2f0ce00fa2e4e36,

title = "International guidelines on the diagnosis and treatment of NUT carcinoma",

abstract = "Nuclear protein in testis (NUT) carcinoma (NC) represents a rare, clinically aggressive cancer defined by pathognomonic NUT Midline Carcinoma Family Member 1 (NUTM1) gene fusions, with bromodomain and extraterminal domain (BET) protein 4 (BRD4)-NUTM1 being the predominant oncogenic driver. Since its description in 1991, gradual advances have clarified the pathologic mechanisms of NC and its diagnostic methods; however, NC treatment remains a significant challenge. Moreover, diagnostic and treatment approaches for this cancer require further validation and standardization. These guidelines were developed by the Chinese Alliance of Research for NC (ChARN) based on current evidence in the literature and incorporate consensus-based input from multiple international experts. They provide comprehensive guidance on NC diagnosis and treatment, covering epidemiology, pathogenesis, diagnostic methods, therapeutic strategies, BET-inhibitor toxicity, palliative care, and prognostic assessment during follow-up. They also emphasize the importance of multidisciplinary team collaboration in NC treatment and recommend prioritizing enrollment in prospective clinical trials for patients. Current mainstays of treatment include surgical resection, radiotherapy, and medical treatment (chemotherapy, targeted therapy, and immunotherapy), although no standard treatment protocol exists. Future research directions include improving diagnostic efficiency, exploring new therapeutic strategies (such as highly selective BET inhibitors, BET-inhibitor combinations, and PROTAC technologies), and recommending basket trials as a research approach for patients with NUTM1 gene fusions.",

keywords = "diagnosis, guideline, NUT carcinoma, treatment",

author = "Yu Zhang and Qi Zhang and Yue Hao and Jia Luo and Yingshi Piao and Wenxian Wang and Zhengbo Song and Ziming Li and Luka Brcic and Aijun Liu and Jinpu Yu and Yasuhiro Tsutani and Wenzhao Zhong and Wenfeng Fang and Zhijie Wang and Shengxiang Ren and Papavassiliou, \{Athanasios G.\} and Yongchang Zhang and Jingjing Liu and Shirong Zhang and Xiuyu Cai and Cangir, \{Ayten Kayi\} and Anwen Liu and Wen Li and Filippo Lococo and Ping Zhan and Hongbing Liu and Tangfeng Lv and Liyun Miao and Lingfeng Min and Helmut Popper and Yu Chen and Jingping Yuan and Feng Wang and Zhansheng Jiang and Gen Lin and Long Huang and Xingxiang Pu and Rongbo Lin and Kalevi Kairemo and Weifeng Liu and Chuangzhou Rao and Dongqing Lv and Zongyang Yu and Ashrafian Leanne and Xiaoyan Li and Chuanhao Tang and Siddique, \{Hifzur R.\} and Chengzhi Zhou and Junping Zhang and Junli Xue and Vishal Shelat and Hui Guo and Qian Chu and Rui Meng and Fatemeh Ardeshir and Jingxun Wu and Rui Zhang and Jin Zhou and Kratzke, \{Robert A.\} and Zhengfei Zhu and Yongheng Li and Hong Qiu and Fan Xia and Fiorella Calabrese and Yang Xia and Mariani, \{Alessandro Wasum\} and Yuanyuan Lu and Xiaofeng Chen and Klein, \{Mark A.\} and Rui Ge and Enyong Dai and Sch{\"o}nthal, \{Axel H.\} and Yu Han and Zhenying Guo and Jian Zhang and Yinghua Ji and Xianbin Liang and Hongmei Zhang and Xuelei Ma and Marco Chiappetta and Xuewen Liu and Salle, \{Francoise Galateau\} and Yu Yao and Malgorzata Szolkowska and Weiwei Pan and Fei Pang and Fan Wu and Watzka, \{Stefan B.\} and Liping Wang and Youcai Zhu and Li Lin and Aparna Sharma and Jianfei Tu and Xinqing Lin and Jing Cai and Ling Xu and Jisheng Li and Xiaodong Jiao and Kainan Li and Zauderer, \{Marjorie G.\} and Jia Wei and Huijing Feng and Lin Wang and Yingying Du and Wang Yao and Elizabeth Dudnik and Xuefei Shi and Xiaomin Niu and Dongmei Yuan and Yanwen Yao and Jianhui Huang and Yue Feng and Yinbin Zhang and Binbin Song and Wenfeng Li and Jianfei Fu and Baine, \{Marina K.\} and Pingli Sun and Hong Wang and Mingxiang Ye and Dong Wang and Zhaofeng Wang and Jing Wu and Yunyun Yang and Yuan Fang and Zhen Wang and Bin Wan and Donglai Lv and Huafei Chen and Shengjie Yang and Jing Kang and Jiatao Zhang and Chao Zhang and Lin Shi and Yina Wang and Sobeih, \{Mohamed Emam\} and Bihui Li and Bin Lian and Lili Mao and Zhang Zhang and Ke Wang and Zhongwu Li and Zhefeng Liu and Nong Yang and Lin Wu and Xiaobing Chen and Gu Jin and Miao Li and Guansong Wang and Marron, \{Thomas U.\} and Jiandong Wang and Sanjay Popat and Meiyu Fang and Yong Fang and Daniel Mansilla and Yuan Li and Xiaojia Wang and Jing Chen and Yiping Zhang and Xixu Zhu and Yi Shen and Shenglin Ma and Mansfield, \{Aaron S.\} and Biyun Wang and Lu Si and Roden, \{Anja C.\} and Gr{\o}nberg, \{Bj{\o}rn H.\} and Yong Song and Shapiro, \{Geoffrey I.\} and French, \{Christopher A.\} and Yuanzhi Lu and Qian Wang and Chunwei Xu",

note = "Publisher Copyright: {\textcopyright} 2025 The Author(s)",

year = "2025",

doi = "10.1016/j.xinn.2025.101068",

language = "英语",

journal = "Innovation",

issn = "2666-6758",

publisher = "Cell Press",

}

Zhang, Y, Zhang, Q, Hao, Y, Luo, J, Piao, Y, Wang, W, Song, Z, Li, Z, Brcic, L, Liu, A, Yu, J, Tsutani, Y, Zhong, W, Fang, W, Wang, Z, Ren, S, Papavassiliou, AG, Zhang, Y, Liu, J, Zhang, S, Cai, X, Cangir, AK, Liu, A, Li, W, Lococo, F, Zhan, P, Liu, H, Lv, T, Miao, L, Min, L, Popper, H, Chen, Y, Yuan, J, Wang, F, Jiang, Z, Lin, G, Huang, L, Pu, X, Lin, R, Kairemo, K, Liu, W, Rao, C, Lv, D, Yu, Z, Leanne, A, Li, X, Tang, C, Siddique, HR, Zhou, C, Zhang, J, Xue, J, Shelat, V, Guo, H, Chu, Q, Meng, R, Ardeshir, F, Wu, J, Zhang, R, Zhou, J, Kratzke, RA, Zhu, Z, Li, Y, Qiu, H, Xia, F, Calabrese, F, Xia, Y, Mariani, AW, Lu, Y, Chen, X, Klein, MA, Ge, R, Dai, E, Schönthal, AH, Han, Y, Guo, Z, Zhang, J, Ji, Y, Liang, X, Zhang, H, Ma, X, Chiappetta, M, Liu, X, Salle, FG, Yao, Y, Szolkowska, M, Pan, W, Pang, F, Wu, F, Watzka, SB, Wang, L, Zhu, Y, Lin, L, Sharma, A, Tu, J, Lin, X, Cai, J, Xu, L, Li, J, Jiao, X, Li, K, Zauderer, MG, Wei, J, Feng, H, Wang, L, Du, Y, Yao, W, Dudnik, E, Shi, X, Niu, X, Yuan, D, Yao, Y, Huang, J, Feng, Y, Zhang, Y, Song, B, Li, W, Fu, J, Baine, MK, Sun, P, Wang, H, Ye, M, Wang, D, Wang, Z, Wu, J, Yang, Y, Fang, Y, Wang, Z, Wan, B, Lv, D, Chen, H, Yang, S, Kang, J, Zhang, J, Zhang, C, Shi, L, Wang, Y, Sobeih, ME, Li, B, Lian, B, Mao, L, Zhang, Z, Wang, K, Li, Z, Liu, Z, Yang, N, Wu, L, Chen, X, Jin, G, Li, M, Wang, G, Marron, TU, Wang, J, Popat, S, Fang, M, Fang, Y, Mansilla, D, Li, Y, Wang, X, Chen, J, Zhang, Y, Zhu, X, Shen, Y, Ma, S, Mansfield, AS, Wang, B, Si, L, Roden, AC, Grønberg, BH, Song, Y, Shapiro, GI, French, CA, Lu, Y, Wang, Q & Xu, C 2025, 'International guidelines on the diagnosis and treatment of NUT carcinoma', Innovation. https://doi.org/10.1016/j.xinn.2025.101068

TY - JOUR

T1 - International guidelines on the diagnosis and treatment of NUT carcinoma

AU - Zhang, Yu

AU - Zhang, Qi

AU - Hao, Yue

AU - Luo, Jia

AU - Piao, Yingshi

AU - Wang, Wenxian

AU - Song, Zhengbo

AU - Li, Ziming

AU - Brcic, Luka

AU - Liu, Aijun

AU - Yu, Jinpu

AU - Tsutani, Yasuhiro

AU - Zhong, Wenzhao

AU - Fang, Wenfeng

AU - Wang, Zhijie

AU - Ren, Shengxiang

AU - Papavassiliou, Athanasios G.

AU - Zhang, Yongchang

AU - Liu, Jingjing

AU - Zhang, Shirong

AU - Cai, Xiuyu

AU - Cangir, Ayten Kayi

AU - Liu, Anwen

AU - Li, Wen

AU - Lococo, Filippo

AU - Zhan, Ping

AU - Liu, Hongbing

AU - Lv, Tangfeng

AU - Miao, Liyun

AU - Min, Lingfeng

AU - Popper, Helmut

AU - Chen, Yu

AU - Yuan, Jingping

AU - Wang, Feng

AU - Jiang, Zhansheng

AU - Lin, Gen

AU - Huang, Long

AU - Pu, Xingxiang

AU - Lin, Rongbo

AU - Kairemo, Kalevi

AU - Liu, Weifeng

AU - Rao, Chuangzhou

AU - Lv, Dongqing

AU - Yu, Zongyang

AU - Leanne, Ashrafian

AU - Li, Xiaoyan

AU - Tang, Chuanhao

AU - Siddique, Hifzur R.

AU - Zhou, Chengzhi

AU - Zhang, Junping

AU - Xue, Junli

AU - Shelat, Vishal

AU - Guo, Hui

AU - Chu, Qian

AU - Meng, Rui

AU - Ardeshir, Fatemeh

AU - Wu, Jingxun

AU - Zhang, Rui

AU - Zhou, Jin

AU - Kratzke, Robert A.

AU - Zhu, Zhengfei

AU - Li, Yongheng

AU - Qiu, Hong

AU - Xia, Fan

AU - Calabrese, Fiorella

AU - Xia, Yang

AU - Mariani, Alessandro Wasum

AU - Lu, Yuanyuan

AU - Chen, Xiaofeng

AU - Klein, Mark A.

AU - Ge, Rui

AU - Dai, Enyong

AU - Schönthal, Axel H.

AU - Han, Yu

AU - Guo, Zhenying

AU - Zhang, Jian

AU - Ji, Yinghua

AU - Liang, Xianbin

AU - Zhang, Hongmei

AU - Ma, Xuelei

AU - Chiappetta, Marco

AU - Liu, Xuewen

AU - Salle, Francoise Galateau

AU - Yao, Yu

AU - Szolkowska, Malgorzata

AU - Pan, Weiwei

AU - Pang, Fei

AU - Wu, Fan

AU - Watzka, Stefan B.

AU - Wang, Liping

AU - Zhu, Youcai

AU - Lin, Li

AU - Sharma, Aparna

AU - Tu, Jianfei

AU - Lin, Xinqing

AU - Cai, Jing

AU - Xu, Ling

AU - Li, Jisheng

AU - Jiao, Xiaodong

AU - Li, Kainan

AU - Zauderer, Marjorie G.

AU - Wei, Jia

AU - Feng, Huijing

AU - Wang, Lin

AU - Du, Yingying

AU - Yao, Wang

AU - Dudnik, Elizabeth

AU - Shi, Xuefei

AU - Niu, Xiaomin

AU - Yuan, Dongmei

AU - Yao, Yanwen

AU - Huang, Jianhui

AU - Feng, Yue

AU - Zhang, Yinbin

AU - Song, Binbin

AU - Li, Wenfeng

AU - Fu, Jianfei

AU - Baine, Marina K.

AU - Sun, Pingli

AU - Wang, Hong

AU - Ye, Mingxiang

AU - Wang, Dong

AU - Wang, Zhaofeng

AU - Wu, Jing

AU - Yang, Yunyun

AU - Fang, Yuan

AU - Wang, Zhen

AU - Wan, Bin

AU - Lv, Donglai

AU - Chen, Huafei

AU - Yang, Shengjie

AU - Kang, Jing

AU - Zhang, Jiatao

AU - Zhang, Chao

AU - Shi, Lin

AU - Wang, Yina

AU - Sobeih, Mohamed Emam

AU - Li, Bihui

AU - Lian, Bin

AU - Mao, Lili

AU - Zhang, Zhang

AU - Wang, Ke

AU - Li, Zhongwu

AU - Liu, Zhefeng

AU - Yang, Nong

AU - Wu, Lin

AU - Chen, Xiaobing

AU - Jin, Gu

AU - Li, Miao

AU - Wang, Guansong

AU - Marron, Thomas U.

AU - Wang, Jiandong

AU - Popat, Sanjay

AU - Fang, Meiyu

AU - Fang, Yong

AU - Mansilla, Daniel

AU - Li, Yuan

AU - Wang, Xiaojia

AU - Chen, Jing

AU - Zhang, Yiping

AU - Zhu, Xixu

AU - Shen, Yi

AU - Ma, Shenglin

AU - Mansfield, Aaron S.

AU - Wang, Biyun

AU - Si, Lu

AU - Roden, Anja C.

AU - Grønberg, Bjørn H.

AU - Song, Yong

AU - Shapiro, Geoffrey I.

AU - French, Christopher A.

AU - Lu, Yuanzhi

AU - Wang, Qian

AU - Xu, Chunwei

PY - 2025

Y1 - 2025

N2 - Nuclear protein in testis (NUT) carcinoma (NC) represents a rare, clinically aggressive cancer defined by pathognomonic NUT Midline Carcinoma Family Member 1 (NUTM1) gene fusions, with bromodomain and extraterminal domain (BET) protein 4 (BRD4)-NUTM1 being the predominant oncogenic driver. Since its description in 1991, gradual advances have clarified the pathologic mechanisms of NC and its diagnostic methods; however, NC treatment remains a significant challenge. Moreover, diagnostic and treatment approaches for this cancer require further validation and standardization. These guidelines were developed by the Chinese Alliance of Research for NC (ChARN) based on current evidence in the literature and incorporate consensus-based input from multiple international experts. They provide comprehensive guidance on NC diagnosis and treatment, covering epidemiology, pathogenesis, diagnostic methods, therapeutic strategies, BET-inhibitor toxicity, palliative care, and prognostic assessment during follow-up. They also emphasize the importance of multidisciplinary team collaboration in NC treatment and recommend prioritizing enrollment in prospective clinical trials for patients. Current mainstays of treatment include surgical resection, radiotherapy, and medical treatment (chemotherapy, targeted therapy, and immunotherapy), although no standard treatment protocol exists. Future research directions include improving diagnostic efficiency, exploring new therapeutic strategies (such as highly selective BET inhibitors, BET-inhibitor combinations, and PROTAC technologies), and recommending basket trials as a research approach for patients with NUTM1 gene fusions.

AB - Nuclear protein in testis (NUT) carcinoma (NC) represents a rare, clinically aggressive cancer defined by pathognomonic NUT Midline Carcinoma Family Member 1 (NUTM1) gene fusions, with bromodomain and extraterminal domain (BET) protein 4 (BRD4)-NUTM1 being the predominant oncogenic driver. Since its description in 1991, gradual advances have clarified the pathologic mechanisms of NC and its diagnostic methods; however, NC treatment remains a significant challenge. Moreover, diagnostic and treatment approaches for this cancer require further validation and standardization. These guidelines were developed by the Chinese Alliance of Research for NC (ChARN) based on current evidence in the literature and incorporate consensus-based input from multiple international experts. They provide comprehensive guidance on NC diagnosis and treatment, covering epidemiology, pathogenesis, diagnostic methods, therapeutic strategies, BET-inhibitor toxicity, palliative care, and prognostic assessment during follow-up. They also emphasize the importance of multidisciplinary team collaboration in NC treatment and recommend prioritizing enrollment in prospective clinical trials for patients. Current mainstays of treatment include surgical resection, radiotherapy, and medical treatment (chemotherapy, targeted therapy, and immunotherapy), although no standard treatment protocol exists. Future research directions include improving diagnostic efficiency, exploring new therapeutic strategies (such as highly selective BET inhibitors, BET-inhibitor combinations, and PROTAC technologies), and recommending basket trials as a research approach for patients with NUTM1 gene fusions.

KW - diagnosis

KW - guideline

KW - NUT carcinoma

KW - treatment

UR - https://www.scopus.com/pages/publications/105013654028

U2 - 10.1016/j.xinn.2025.101068

DO - 10.1016/j.xinn.2025.101068

M3 - 文献综述

AN - SCOPUS:105013654028

SN - 2666-6758

JO - Innovation

JF - Innovation

M1 - 101068

ER -

International guidelines on the diagnosis and treatment of NUT carcinoma

Abstract

Keywords

UN SDGs

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