Brain Tumors in NF1 Children: Influence on Neurocognitive and Behavioral Outcome
1
Developmental Neurology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11, 20133 Milan, Italy
2
Neuroradiology Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Via Celoria 11, 20133 Milan, Italy
*
Author to whom correspondence should be addressed.
†
These authors contributed equally to this work.
Cancers 2019, 11(11), 1772; https://doi.org/10.3390/cancers11111772
Received: 30 September 2019
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Revised: 30 October 2019
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Accepted: 5 November 2019
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Published: 11 November 2019
(This article belongs to the Special Issue New Insights into Neurofibromatosis)
Neurofibromatosis type-1 (NF1) is a monogenic tumor-predisposition syndrome creating a wide variety of cognitive and behavioral abnormalities, such as decrease in cognitive functioning, deficits in visuospatial processing, attention, and social functioning. NF1 patients are at risk to develop neurofibromas and other tumors, such as optic pathway gliomas and other tumors of the central nervous system. Few studies have investigated the impact of an additional diagnosis of brain tumor on the cognitive outcome of children with NF1, showing unclear results and without controlling by the effect of surgery, radio- or chemotherapy. In the present mono-institutional study, we compared the behavioral and cognitive outcomes of 26 children with neurofibromatosis alone (NF1) with two age-matched groups of 26 children diagnosed with NF1 and untreated optic pathway glioma (NF1 + OPG) and 19 children with NF1 and untreated other central nervous system tumors (NF1 + CT). NF1 + CT and NF1 + OPG showed significantly impaired cognitive abilities compared to NF1 group, with weaknesses in visuo-spatial abilities, visual scanning and verbal working memory, while general verbal abilities are preserved. Moreover, NF1 + OPG patients present more frequent internalizing problems and increased oppositional-deviant behaviors. These results suggest that the co-diagnosis of a brain tumor in NF1 children may partially worsen the cognitive and emotional outcome.
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Keywords:
neurofibromatosis type-1; children; optic pathways glioma; brain tumor; cognitive-behavioral outcome
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MDPI and ACS Style
Taddei, M.; Erbetta, A.; Esposito, S.; Saletti, V.; Bulgheroni, S.; Riva, D. Brain Tumors in NF1 Children: Influence on Neurocognitive and Behavioral Outcome. Cancers 2019, 11, 1772. https://doi.org/10.3390/cancers11111772
AMA Style
Taddei M, Erbetta A, Esposito S, Saletti V, Bulgheroni S, Riva D. Brain Tumors in NF1 Children: Influence on Neurocognitive and Behavioral Outcome. Cancers. 2019; 11(11):1772. https://doi.org/10.3390/cancers11111772
Chicago/Turabian StyleTaddei, Matilde, Alessandra Erbetta, Silvia Esposito, Veronica Saletti, Sara Bulgheroni, and Daria Riva. 2019. "Brain Tumors in NF1 Children: Influence on Neurocognitive and Behavioral Outcome" Cancers 11, no. 11: 1772. https://doi.org/10.3390/cancers11111772
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