Pleuropulmonary blastoma in children and adolescents: The EXPeRT/PARTNER diagnostic and therapeutic recommendations.
PARTNER
children
pleuropulmonary blastoma
therapeutic recommendations
very rare tumors
Journal
Pediatric blood & cancer
ISSN: 1545-5017
Titre abrégé: Pediatr Blood Cancer
Pays: United States
ID NLM: 101186624
Informations de publication
Date de publication:
06 2021
06 2021
Historique:
revised:
15
03
2021
received:
14
02
2021
accepted:
16
03
2021
pubmed:
8
4
2021
medline:
6
5
2022
entrez:
7
4
2021
Statut:
ppublish
Résumé
Pleuropulmonary blastoma (PPB) is a rare cancer occurring mainly during early childhood and often associated with germline DICER1 mutations. It is classified by the macroscopic appearance into three interrelated clinico-pathologic entities on a developmental continuum. Complete tumor resection is a main prognostic factor and can be performed at diagnosis or after neoadjuvant treatment that includes chemotherapy and in some cases radiotherapy. Optimal modalities of neo- or adjuvant treatments can be challenging taking into account potential long-term toxicities in this young population. This paper presents the recommendations for diagnosis and treatment of children and adolescents with PPB elaborated by the European Cooperative Study Group for Pediatric Rare Tumors (EXPeRT) within the European Union-funded project PARTNER (Paediatric Rare Tumours Network - European Registry).
Identifiants
pubmed: 33826235
doi: 10.1002/pbc.29045
pmc: PMC9813943
mid: NIHMS1858645
doi:
Substances chimiques
DICER1 protein, human
EC 3.1.26.3
Ribonuclease III
EC 3.1.26.3
DEAD-box RNA Helicases
EC 3.6.4.13
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
e29045Subventions
Organisme : NCI NIH HHS
ID : R37 CA244940
Pays : United States
Informations de copyright
© 2021 Wiley Periodicals LLC.
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