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NUT carcinoma in children, adolescents and young adults.
Lemelle, Lauriane; Moya-Plana, Antoine; Dumont, Benoît; Fresneau, Brice; Laprie, Anne; Claude, Line; Deneuve, Sophie; Cordero, Camille; Pierron, Gaelle; Couloigner, Vincent; Bernard, Sophie; Cardoen, Liesbeth; Brisse, Hervé J; Jehanno, Nina; Metayer, Lucy; Fréneaux, Paul; Helfre, Sylvie; Kolb, Fréderic; Thariat, Juliette; Réguerre, Yves; Orbach, Daniel.
  • Lemelle L; PSL University, Institut Curie, SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), 75005 Paris, France; University of Paris, Paris, France. Electronic address: lauriane.lemelle@curie.fr.
  • Moya-Plana A; Paris-Saclay University, Gustave Roussy, Surgical and Interventional Department, 94805 Villejuif, France.
  • Dumont B; Institute for Paediatric Haematology and Oncology, Leon Bérard Cancer Centre, 69008 Lyon, France.
  • Fresneau B; Université Paris-Saclay, Gustave Roussy, Department of Pediatric oncology, 94805 Villejuif, France.
  • Laprie A; University Institute of Cancer Toulouse-Oncopôle, Department of Radiation Oncology, 31100 Toulouse, France.
  • Claude L; Léon Bérard Cancer Center, Radiation Oncology department, 69008 Lyon, France.
  • Deneuve S; Centre Léon Bérard, Oncologic Surgery Department, 69008 Lyon, France.
  • Cordero C; PSL University, Institut Curie, SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), 75005 Paris, France.
  • Pierron G; Institut Curie, PSL Research University, Genetics Unit, Department of Tumor Biology, 75005 Paris, France.
  • Couloigner V; Assistance Publique-Hôpitaux de Paris, hôpital Necker Enfants-Malades, département de chirurgie de la tête et du cou, 75015 Paris, France.
  • Bernard S; Assistance Publique-Hôpitaux de Paris, hôpital Robert Debré, département de chirurgie de la tête et du cou, 75019 Paris, France.
  • Cardoen L; Institut Curie, département d'imagerie, Paris, France.
  • Brisse HJ; Institut Curie, département d'imagerie, Paris, France.
  • Jehanno N; Institut Curie, département de médecine nucléaire, Paris, France.
  • Metayer L; PSL University, Institut Curie, SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), 75005 Paris, France.
  • Fréneaux P; Institut Curie, département de médecine diagnostique et théranostique, 75005 Paris, France.
  • Helfre S; Institut Curie, département de radiothérapie, 75005 Paris, France.
  • Kolb F; University of California, Division of Plastic Surgery, Department of Surgery, San Diego California, USA.
  • Thariat J; Baclesse Cancer Center, Radiation Oncology Department, 14000 Caen, France.
  • Réguerre Y; CHU de Saint Denis, service d'oncologie et d'hématologie pédiatrique, 97400 Saint-Denis, Reunion.
  • Orbach D; PSL University, Institut Curie, SIREDO Oncology Center (Care, Innovation and Research for Children, Adolescents and Young Adults with Cancer), 75005 Paris, France.
Bull Cancer ; 109(4): 491-504, 2022 Apr.
Article en En | MEDLINE | ID: mdl-35428456
ABSTRACT

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.
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Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Proteínas Nucleares / Carcinoma Tipo de estudio: Prognostic_studies Límite: Adolescent / Adult / Child / Humans Idioma: En Año: 2022 Tipo del documento: Article

Texto completo: 1 Banco de datos: MEDLINE Asunto principal: Proteínas Nucleares / Carcinoma Tipo de estudio: Prognostic_studies Límite: Adolescent / Adult / Child / Humans Idioma: En Año: 2022 Tipo del documento: Article