Low-Grade Gliomas in Patients with Noonan Syndrome: Case-Based Review of the Literature.

Noonan syndrome cancer predisposition everolimus mTOR signaling pediatric brain tumor

Journal

Diagnostics (Basel, Switzerland)
ISSN: 2075-4418
Titre abrégé: Diagnostics (Basel)
Pays: Switzerland
ID NLM: 101658402

Informations de publication

Date de publication:
12 Aug 2020
Historique:
received: 19 07 2020
revised: 08 08 2020
accepted: 10 08 2020
entrez: 19 8 2020
pubmed: 19 8 2020
medline: 19 8 2020
Statut: epublish

Résumé

Noonan syndrome (NS) is a congenital autosomic dominant condition characterized by a variable spectrum from a clinical and genetical point of view. Germline mutations in more than ten genes involved in RAS-MAPK signal pathway have been demonstrated to cause the disease. An higher risk for leukemia and solid malignancies, including brain tumors, is related to NS. A review of the published literature concerning low grade gliomas (LGGs) in NS is presented. We described also a 13-year-old girl with NS associated with a recurrent mutation in

Identifiants

pubmed: 32806529
pii: diagnostics10080582
doi: 10.3390/diagnostics10080582
pmc: PMC7460327
pii:
doi:

Types de publication

Case Reports

Langues

eng

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Auteurs

Mariachiara Lodi (M)

Department of Paediatric Haematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Luigi Boccuto (L)

School of Nursing, College of Behavioral, Social and Health Sciences, Clemson University, Clemson, SC 29634, USA.
JC Self Research Institute of the Greenwood Genetic Center, Greenwood, SC 29646, USA.

Andrea Carai (A)

Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Antonella Cacchione (A)

Department of Paediatric Haematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Evelina Miele (E)

Department of Paediatric Haematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Giovanna Stefania Colafati (GS)

Neuroradiology Unit, Department of Imaging, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Francesca Diomedi Camassei (F)

Department of Laboratories, Pathology Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Luca De Palma (L)

Neurology Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Alessandro De Benedictis (A)

Neurosurgery Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Elisabetta Ferretti (E)

Neurology Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Giuseppina Catanzaro (G)

Department of Experimental Medicine, Sapienza University, 00161 Rome, Italy.

Agnese Pò (A)

Department of Molecular Medicine, Sapienza University, 00161 Rome, Italy.

Alessandro De Luca (A)

Medical Genetics Division, Fondazione IRCCS-Casa Sollievo della Sofferenza, 71043 San Giovanni Rotondo, Italy.

Martina Rinelli (M)

Laboratory of Medical Genetics, Department of Laboratories, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Francesca Romana Lepri (FR)

Laboratory of Medical Genetics, Department of Laboratories, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Emanuele Agolini (E)

Laboratory of Medical Genetics, Department of Laboratories, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Marco Tartaglia (M)

Genetics and Rare Diseases Research Division, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Franco Locatelli (F)

Department of Paediatric Haematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.
Department of Pediatric Hematology and Oncology Cell and Gene Therapy, Bambino Gesù Hospital, IRCCS, University La Sapienza, 00165 Rome, Italy.

Angela Mastronuzzi (A)

Department of Paediatric Haematology/Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome, Italy.

Classifications MeSH