Update on childhood craniopharyngiomas.
Craniopharyngioma location impacts treatment approach. Imaging advances allow for better anatomical localization, which can help determine the best surgical plan. Recent discoveries have also led to a better understanding of craniopharyngioma development and potential treatments. This review includes publications January 2015 through March 2016 and prior key reports.



Recent findings confirm that third ventricular and hypothalamic involvement are associated with highest risk of hypothalamic dysfunction after surgery. Both presentation and MRI can aid in presurgical grading to try to limit development of hypothalamic obesity, somnolence, neurocognitive dysfunction, decreased quality of life, and other morbidities. Targeted therapies may also prove useful in avoiding treatment complications. In total, 14-50% of adult-onset craniopharyngioma are papillary; the majority with a mutation in exon 3 of BRAF and may respond to BRAF inhibitors and mitogen-activated protein kinase inhibitors. The remaining adult-onset and majority of childhood-onset are adamantinomatous; often with mutations in CTNNB1, which encodes β-catenin, leading to overactivation of the WNT signaling pathway.



Significant morbidities are associated with craniopharyngioma. Targeted medical therapies are on the horizon. Until that time, the surgical approach and decision for radiation therapy should be chosen to limit long-term sequelae.
Division of Endocrinology, Boston Children's Hospital, Harvard Medical School, Boston, MA.
Laurie E
LE
Cohen
D002648:Child; D003397:Craniopharyngioma / Q000235:genetics / Q000473:pathology / Q000601:surgery; D006801:Humans; D010911:Pituitary Neoplasms / Q000235:genetics / Q000473:pathology / Q000601:surgery