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Article ; Online: Are B7-H3 CAR-T cells the future universal treatment for pediatric brain tumors?

Bourdeaut, Franck

2021 Volume 23, Issue 6, Page(s) 872–873

MeSH term(s)	Antigens, Surface ; Brain Neoplasms/therapy ; Child ; Humans ; Immunotherapy, Adoptive ; Receptors, Chimeric Antigen ; T-Lymphocytes
Chemical Substances	Antigens, Surface ; Receptors, Chimeric Antigen
Language	English
Publishing date	2021-03-23
Publishing country	England
Document type	Editorial ; Comment
ZDB-ID	2028601-6
ISSN	1523-5866 ; 1522-8517
ISSN (online)	1523-5866
ISSN	1522-8517
DOI	10.1093/neuonc/noab063
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Article ; Online: Right to know and right to ignore in paediatric oncogenetics: Identifying biological causes, or seeking for meaning?

Bourdeaut, Franck

European journal of cancer (Oxford, England : 1990)

2016 Volume 63, Page(s) 53–54

Language	English
Publishing date	2016-08
Publishing country	England
Document type	Editorial
ZDB-ID	82061-1
ISSN	1879-0852 ; 0277-5379 ; 0959-8049 ; 0964-1947
ISSN (online)	1879-0852
ISSN	0277-5379 ; 0959-8049 ; 0964-1947
DOI	10.1016/j.ejca.2016.04.021
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Zs.A 456: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 583: Show issues

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Article: Prédispositions génétiques aux tumeurs osseuses.

Bourdeaut, Franck

Soins. Pediatrie, puericulture

2013 , Issue 270, Page(s) 19

Title translation	Genetic predisposition to bone tumors.
MeSH term(s)	Adolescent ; Bone Neoplasms/genetics ; Child ; Genetic Predisposition to Disease ; Humans ; Osteosarcoma/genetics ; Sarcoma, Ewing/genetics
Language	French
Publishing date	2013-01
Publishing country	France
Document type	Journal Article
ZDB-ID	1234484-9
ISSN	2214-9325 ; 1259-4792
ISSN (online)	2214-9325
ISSN	1259-4792
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Zs.B 2788: Show issues

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Article ; Online: Genetic predisposition to medulloblastomas: just follow the tumour genome.

Bourdeaut, Franck / Delattre, Olivier

The Lancet. Oncology

2018 Volume 19, Issue 6, Page(s) 722–723

Language	English
Publishing date	2018-05-09
Publishing country	England
Document type	Journal Article ; Comment
ZDB-ID	2049730-1
ISSN	1474-5488 ; 1470-2045
ISSN (online)	1474-5488
ISSN	1470-2045
DOI	10.1016/S1470-2045(18)30289-4
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Zs.A 5696: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (2.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Embryonal tumors of the central nervous system.

Pagès, Mélanie / Masliah-Planchon, Julien / Bourdeaut, Franck

Current opinion in oncology

2020 Volume 32, Issue 6, Page(s) 623–630

Abstract: Purpose of review: This review aims to give an update on histopathological, molecular and clinical features of central nervous system (CNS) 'embryonal' tumors.: Recent findings: The taxonomy of previously called 'CNS primitive neuroectodermal tumor' ( ...

Abstract	Purpose of review: This review aims to give an update on histopathological, molecular and clinical features of central nervous system (CNS) 'embryonal' tumors. Recent findings: The taxonomy of previously called 'CNS primitive neuroectodermal tumor' (CNS PNET) has been deeply modified since the discovery of specific molecular profiles for each various sub-entity of these rare, mainly pediatric, tumors. The term 'embryonal tumors' now refers to medulloblastomas, atypical teratoid rhabdoid tumors (AT/RT) and other rare entities, defined by their specific histopathological features together with expression-based or methylation-based profiling; specific gene mutations or fusions characterize some tumor types. In addition, the compilation of large series of molecular data has allowed to dissecting several of these tumor types in molecular subgroups, increasing the number of tumor entities, and leading to an amazingly complex nosology of rare-to-extremely rare malignancies. This rarity precludes from having strong evidence-based therapeutic recommendations, although international efforts are conducted to define the best treatment strategies. Summary: Embryonal tumors now correspond to molecularly well defined entities, which deserve further international collaborations to specify their biology and the appropriate burden of treatment, in order to minimize the long-term side-effects of treatment of these overall rare and severe diseases of childhood.
MeSH term(s)	Central Nervous System Neoplasms/pathology ; Central Nervous System Neoplasms/therapy ; Gene Fusion ; Humans ; Medulloblastoma/pathology ; Medulloblastoma/therapy ; Mutation ; Neoplasms, Germ Cell and Embryonal/pathology ; Neoplasms, Germ Cell and Embryonal/therapy ; Rhabdoid Tumor/pathology ; Rhabdoid Tumor/therapy ; Teratoma/pathology ; Teratoma/therapy
Language	English
Publishing date	2020-09-01
Publishing country	United States
Document type	Journal Article ; Review
ZDB-ID	1049384-0
ISSN	1531-703X ; 1040-8746
ISSN (online)	1531-703X
ISSN	1040-8746
DOI	10.1097/CCO.0000000000000686
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Article ; Online: Checkpoint Immunotherapy in Pediatrics: Here, Gone, and Back Again.

Long, Adrienne H / Morgenstern, Daniel A / Leruste, Amaury / Bourdeaut, Franck / Davis, Kara L

American Society of Clinical Oncology educational book. American Society of Clinical Oncology. Annual Meeting

2022 Volume 42, Page(s) 1–14

Abstract: The role of immune checkpoint inhibitors (ICIs) in the treatment of pediatric cancers continues to evolve. Such therapies function by augmenting existing antitumor T-cell responses that have been rendered ineffective by inhibitory pathways. Although ICIs ...

Abstract	The role of immune checkpoint inhibitors (ICIs) in the treatment of pediatric cancers continues to evolve. Such therapies function by augmenting existing antitumor T-cell responses that have been rendered ineffective by inhibitory pathways. Although ICIs have proven highly effective for adult cancers, initial phase I/II clinical trials using single-agent ICIs against unselected pediatric cancers have been overall disappointing. With the exception of pediatric classic Hodgkin lymphoma, responses to ICIs have been infrequent, likely stemming from an inherent difference in the immunogenicity of childhood cancers, which, on average, have far fewer neoantigens than adult cancers. Recently, however, hope has reemerged that certain subsets of children with cancer may benefit from ICI therapies. In preliminary studies, patients with both pediatric hypermutated and SMARCB1-deficient cancers have had impressive responses to ICI therapies, likely as a result of underlying biologies that enhance neoantigen expression and tumoral inflammation. Dedicated trials are ongoing to fully evaluate the efficacy of ICIs for patients with these subsets of pediatric cancer.
MeSH term(s)	Child ; Hodgkin Disease ; Humans ; Immune Checkpoint Inhibitors ; Immunologic Factors ; Immunotherapy ; Pediatrics
Chemical Substances	Immune Checkpoint Inhibitors ; Immunologic Factors
Language	English
Publishing date	2022-05-17
Publishing country	United States
Document type	Journal Article
ZDB-ID	2431126-1
ISSN	1548-8756 ; 1548-8748
ISSN (online)	1548-8756
ISSN	1548-8748
DOI	10.1200/EDBK_349799
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Article ; Online: Cancer chez un enfant porteur de handicap intellectuel : questionnement et enjeux éthiques.

Auvrignon, Anne / Vialle, Guénola / Gérard, Delphine / Pommier de Santi, Claire / Bourdeaut, Franck / Davous, Dominique

Bulletin du cancer

2021 Volume 108, Issue 9, Page(s) 827–836

Abstract: The Parents and Caregivers group in the face of ethics in pediatrics of the Île-de-France Ethics Area wondered about the association of the words Disability and Cancer by focusing on the study of the course of children with intellectual disability, ... ...

Title translation	Cancer in children with intellectual disabilities: Questioning and ethical issues.
Abstract	The Parents and Caregivers group in the face of ethics in pediatrics of the Île-de-France Ethics Area wondered about the association of the words Disability and Cancer by focusing on the study of the course of children with intellectual disability, treated for cancer. These situations are exceptional, the number of cases in France must not be more than fifty per year. We gathered the testimony of five families of children using a semi-directive survey taking up the journey from birth, announcement of the handicap, the diagnosis of cancer and its treatment. The verbatim show that each story is unique and rich in lessons, despite the feeling of "double penalty": "He did not deserve this, a handicap plus cancer is a lot for one person", "the shot moreover." A healthcare team was also interviewed and raised an additional question: "First, the double penalty… then, what's the point?" Through these testimonies, we sought to question the ethical principles of care, which can be shaken up in these extraordinary supported.
MeSH term(s)	Agenesis of Corpus Callosum/diagnosis ; Agenesis of Corpus Callosum/psychology ; Bioethical Issues ; Caregivers ; Child ; Child, Preschool ; Clinical Decision-Making/ethics ; Disabled Children/statistics & numerical data ; Down Syndrome/diagnosis ; Down Syndrome/psychology ; Family/psychology ; Family Relations ; Female ; Fragile X Syndrome/diagnosis ; Fragile X Syndrome/psychology ; France/epidemiology ; Humans ; Infant ; Intellectual Disability/diagnosis ; Intellectual Disability/epidemiology ; Intellectual Disability/psychology ; Male ; Neoplasms/diagnosis ; Neoplasms/epidemiology ; Neoplasms/psychology ; Neoplasms/therapy ; Parents/psychology ; Personal Autonomy ; Qualitative Research ; Truth Disclosure
Language	French
Publishing date	2021-07-30
Publishing country	France
Document type	Journal Article
ZDB-ID	213270-9
ISSN	1769-6917 ; 0007-4551
ISSN (online)	1769-6917
ISSN	0007-4551
DOI	10.1016/j.bulcan.2021.07.001
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Article ; Online: Déficience intellectuelle et cancer chez l’enfant : analyse des pratiques concernant le choix du traitement.

Dangles, Marie-Thérèse / Davous, Dominique / Vialle, Guénola / Auvrignon, Anne / Angellier, Elisabeth / Bourdeaut, Franck

Bulletin du cancer

2021 Volume 108, Issue 9, Page(s) 813–826

Abstract: Aim: The aim was to describe and to analyze the ethics of decision-making in situations involving children with intellectual disability and cancer, from the referent-doctor's point-of-view, in pediatric oncology units in France.: Methods: ... ...

Title translation	Intellectual disability and cancer in children: An analysis of the decision-making process.
Abstract	Aim: The aim was to describe and to analyze the ethics of decision-making in situations involving children with intellectual disability and cancer, from the referent-doctor's point-of-view, in pediatric oncology units in France. Methods: Pediatricians working in pediatric oncology units were interviewed through an online questionnaire and a semi-directive interview was systematically proposed. We analyzed the ethical issues that arose during the process of decision-making and we made suggestions in order to address them. Results: Sixteen doctors reported twenty-one clinical cases. Of these cases, one third of the children had a change in their oncologic treatment, with a risk of pejorative outcome on the prognosis. Despite the fact that ethical issues appeared in 80 % of the cases, there were few consultations with ethical committees. Decision-making process showed no difference compared to children without intellectual disability, thus raising ethical issues in the medical team. Our study showed discrepancy between frequently reported ethical issues, high consensus rate regarding treatment decision and lack of consultation with ethical committees. Discussion: We propose three steps to guide the decision-making process in situations involving children with intellectual disability and cancer: 1/deeper understanding of the child through reinforced interactions with their caregivers, 2/better cross-boundary discussions, to improve the effectiveness of the multidisciplinary staff, and 3/more systematic ethical committees consultation.
MeSH term(s)	Adolescent ; Adult ; Bioethical Issues ; Child ; Child, Preschool ; Clinical Decision-Making/ethics ; Clinical Decision-Making/methods ; Consensus ; Decision Support Techniques ; Disabled Children ; Ethics Committees, Clinical/statistics & numerical data ; Female ; France ; Health Care Surveys ; Humans ; Infant ; Intellectual Disability/complications ; Male ; Neoplasms/complications ; Neoplasms/pathology ; Neoplasms/therapy ; Palliative Care ; Patient Care Team ; Risk Assessment ; Truth Disclosure ; Young Adult
Language	French
Publishing date	2021-06-24
Publishing country	France
Document type	Journal Article
ZDB-ID	213270-9
ISSN	1769-6917 ; 0007-4551
ISSN (online)	1769-6917
ISSN	0007-4551
DOI	10.1016/j.bulcan.2021.02.013
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Article ; Online: A comprehensive histomolecular characterization of meningioangiomatosis: Further evidence for a precursor neoplastic lesion.

Tauziède-Espariat, Arnault / Masliah-Planchon, Julien / Sievers, Philipp / Sahm, Felix / Dangouloff-Ros, Volodia / Boddaert, Nathalie / Hasty, Lauren / Aboubakr, Oumaima / Métais, Alice / Chrétien, Fabrice / Roux, Alexandre / Pallud, Johan / Blauwblomme, Thomas / Beccaria, Kévin / Bourdeaut, Franck / Puget, Stéphanie / Varlet, Pascale

Brain pathology (Zurich, Switzerland)

2024 , Page(s) e13259

Abstract: Meningioangiomatosis (MAM) remains a poorly understood lesion responsible for epileptic disease. In the past, MAM was primarily described in the context of neurofibromatosis type 2 before being mainly reported sporadically. Moreover, the malformative or ... ...

Abstract	Meningioangiomatosis (MAM) remains a poorly understood lesion responsible for epileptic disease. In the past, MAM was primarily described in the context of neurofibromatosis type 2 before being mainly reported sporadically. Moreover, the malformative or tumoral nature is still debated. Because a subset of MAM are associated with meningiomas, some authors argue that MAM corresponds to an infiltration pattern of these tumors. For these reasons, MAM has not been added to the World Health Organization (WHO) Classification of Central Nervous System Tumors as a specific entity. In the present study, we characterized a series of pure MAM (n = 7) and MAM associated with meningiomas (n = 4) using histopathology, immunohistochemistry, genetic (fluorescent in situ and DNA sequencing analyses), and epigenetic (DNA-methylation profiling) data. We evidenced two distinct morphological patterns: MAM with a fibroblastic-like pattern having few lesional cells, and MAM with a more cellular pattern. A subset was associated with the genetic alterations previously reported in meningiomas (such as a KMT2C mutation and a hemizygous deletion of chromosome 22q including the NF2 gene). The DNA-methylation profile, using a t-distributed stochastic neighbor embedding analysis, evidenced that MAM (pure or associated with meningiomas) clustered in a separate group from pediatric meningiomas. The present results seem to suggest that MAM represents a neoplastic lesion and encourage the further study of similar additional series so that it may be included in a future WHO classification.
Language	English
Publishing date	2024-04-02
Publishing country	Switzerland
Document type	Journal Article
ZDB-ID	1051484-3
ISSN	1750-3639 ; 1015-6305
ISSN (online)	1750-3639
ISSN	1015-6305
DOI	10.1111/bpa.13259
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Article ; Online: Immune responses in genomically simple SWI/SNF-deficient cancers.

Leruste, Amaury / Chauvin, Céline / Pouponnot, Celio / Bourdeaut, Franck / Waterfall, Joshua J / Piaggio, Eliane

Cancer

2020 Volume 127, Issue 2, Page(s) 172–180

MeSH term(s)	Animals ; Combined Modality Therapy/methods ; DNA Helicases/deficiency ; DNA Helicases/genetics ; DNA-Binding Proteins/deficiency ; DNA-Binding Proteins/genetics ; Disease Models, Animal ; Humans ; Immune Checkpoint Inhibitors/therapeutic use ; Immunity ; Mice ; Mutation ; Neoplasms/drug therapy ; Neoplasms/immunology ; Neoplasms/metabolism ; Neoplasms/radiotherapy ; Nuclear Proteins/deficiency ; Nuclear Proteins/genetics ; Polymorphism, Single Nucleotide ; SMARCB1 Protein/deficiency ; SMARCB1 Protein/genetics ; Transcription Factors/deficiency ; Transcription Factors/genetics ; Treatment Outcome
Chemical Substances	DNA-Binding Proteins ; Immune Checkpoint Inhibitors ; Nuclear Proteins ; PBRM1 protein, human ; SMARCB1 Protein ; SMARCB1 protein, human ; Transcription Factors ; SMARCA4 protein, human (EC 3.6.1.-) ; DNA Helicases (EC 3.6.4.-)
Keywords	covid19
Language	English
Publishing date	2020-10-20
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1429-1
ISSN	1097-0142 ; 0008-543X ; 1934-662X
ISSN (online)	1097-0142
ISSN	0008-543X ; 1934-662X
DOI	10.1002/cncr.33172
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