[Antineoplastic drug-induced nausea and vomiting in pediatric onco-hematology: 2022: Guidelines from the supportive care committee of the French Society of Childhood Cancer (SFCE)]. / Nausées-vomissements induits par les traitements anticancéreux (NVITAC) en onco-hématologie pédiatrique : recommandations 2022 du Comité soins de support de la SFCE.

Nausea and vomiting induced by cancer treatment (CINV) remain one of the most common and feared side effects in children despite the use of new drugs to prevent them. The existing recommendations for the prophylaxis and treatment of CINV are based on adult patients in Anglo-Saxon societies. Based on a recent review of the literature, we focused on specific pediatric issues in order to offer recommendations validated by the supportive care committee of the French society for childhood cancer.

Mosaic PTEN alteration in the neural crest during embryogenesis results in multiple nervous system hamartomas.

The contribution of mosaic alterations to tumors of the nervous system and to non-malignant neurological diseases has been unmasked thanks to the development of Next Generation Sequencing (NGS) technologies. We report here the case of a young patient without any remarkable familial medical history who was first referred at 7 years of age, for an autism spectrum disorder (ASD) of Asperger type, not associated with macrocephaly. The patient subsequently presented at 10 years of age with multiple nodular lesions located within the trigeminal, facial and acoustic nerve ganglia and at the L3 level. Histological examination of this latter lesion revealed a glioneuronal hamartoma, exhibiting heterogeneous PTEN immunoreactivity, astrocyte and endothelial cell nuclei expressing PTEN, but not ganglion cells. NGS performed on the hamartoma allowed the detection of a PTEN pathogenic variant in 30% of the reads. The presence of this variant in the DNA extracted from blood and buccal swabs in 3.5 and 11% of the NGS reads, respectively, confirmed the mosaic state of the PTEN variant. The anatomical distribution of the lesions suggests that the mutational event affecting PTEN occurred in neural crest progenitors, thus explaining the absence of macrocephaly. This report shows that mosaic alteration of PTEN may result in multiple central and peripheral nervous system hamartomas and that the presence of such alteration should be considered in patients with multiple nervous system masses, even in the absence of cardinal features of PTEN hamartoma tumor syndrome, especially macrocephaly.