NUT carcinoma in children, adolescents and young adults

被引：5

作者：

Lemelle, Lauriane ^{[1
,2
]}

Moya-Plana, Antoine ^{[3
]}

Dumont, Benoit ^{[4
]}

Fresneau, Brice ^{[5
]}

Laprie, Anne ^{[6
]}

Claude, Line ^{[7
]}

Deneuve, Sophie ^{[8
]}

Cordero, Camille ^{[1
]}

Pierron, Gaelle ^{[9
]}

Couloigner, Vincent ^{[10
]}

Bernard, Sophie ^{[11
]}

Cardoen, Liesbeth ^{[12
]}

Brisse, Nerve J. ^{[12
]}

Jehanno, Nina ^{[13
]}

Metayer, Lucy ^{[1
]}

Freneaux, Paul ^{[14
]}

Helfre, Sylvie ^{[15
]}

Kolb, Frederic ^{[16
]}

Thariat, Juliette ^{[17
]}

Reguerre, Yves ^{[18
]}

Orbach, Daniel ^{[1
]}

机构：

[1] PSL Univ, Inst Curie, SIREDO Oncol Ctr Care Innovat & Res Children Adol, F-75005 Paris, France

[2] Univ Paris, Paris, France

[3] Paris Saclay Univ, Gustave Roussy, Surg & Intervent Dept, F-94805 Villejuif, France

[4] Leon Berard Canc Ctr, Inst Paediat Haematol & Oncol, F-69008 Lyon, France

[5] Univ Paris Saclay, Dept Pediat Oncol, Gustave Roussy, F-94805 Villejuif, France

[6] Univ Inst Canc Toulouse Oncopole, Dept Radiat Oncol, F-31100 Toulouse, France

[7] Leon Berard Canc Ctr, Radiat Oncol Dept, F-69008 Lyon, France

[8] Ctr Leon Berard, Oncol Surg Dept, F-69008 Lyon, France

[9] PSL Res Univ, Inst Curie, Dept Tumor Biol, Genet Unit, F-75005 Paris, France

[10] Hop Necker Enfants Malad, Assistance Publ Hop Paris, Dept Chirurg Tete & Cou, F-75015 Paris, France

[11] Hop Robert Debre, Assistance Publ Hop Paris, Dept Chirurg Tete & Cou, F-75019 Paris, France

[12] Inst Curie, Dept Imagerie, Paris, France

[13] Inst Curie, Dept Med Nucl, Paris, France

[14] Inst Curie, Dept Med Diagnost & Theranost, F-75005 Paris, France

[15] Inst Curie, Dept Radiotherapie, F-75005 Paris, France

[16] Univ Calif San Diego, Div Plast Surg, Dept Surg, San Diego, CA 92103 USA

[17] Badesse Canc Ctr, Radiat Oncol Dept, F-14000 Caen, France

[18] CHU St Denis, Serv Oncol & Hematol Pediat, F-97400 St Denis, Reunion, France

来源：

BULLETIN DU CANCER | 2022年 / 109卷 / 04期

关键词：

NUT carcinoma; Rare tumour; Midline carcinoma; Targeted therapies; Children; Bromodomain and; Extra-Terminal (BET); protein; Histone deacetylase; HISTONE DEACETYLASE INHIBITORS; MIDLINE CARCINOMA; TUMOR-GROWTH; BRD4; SARCOMA; TRANSCRIPTION; DIAGNOSIS; PROTEINS; EFFICACY; FEATURES;

D O I：

10.1016/j.bulcan.2022.01.015

中图分类号：

R73 [肿瘤学];

学科分类号：

100214 ;

摘要：

Background > NUT carcinoma (NC), defined by the presence of the NUTM1 rearrangement, is an aggressive tumour associated with poor prognosis. This rare cancer is underdiagnosed and difficult to treat. Objective and methods > The primary objective of this review is to describe the clinical, radiological and laboratory features of NC in young patients. The secondary objective is to propose a consensual strategy for the French very Rare Tumour group (FRACTURE group). Results > NUT-specific antibody immunostaining in cases of undifferentiated or poorly differentiated carcinoma may demonstrate the specific NUT gene rearrangement. NCs are frequently advanced stage at diagnosis and the outcome remains poor despite a global strategy that generally includes conventional combination chemotherapy with wide local therapy (surgery, radiotherapy). Chemosensitivity is frequently only transient. Conclusion > Recent data have shown that new targeted drugs (histone deacetylase and bromodomain and extra-terminal protein inhibitors) are promising, but their role has yet to be evaluated in NC. Centralized data review is necessary to improve our knowledge of paediatric NC. We propose a multimodal strategy based on published data and their personal experience.

引用

页码：491 / 504

页数：14

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