2018
DOI: 10.3171/2018.3.focus1861
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Craniopharyngioma: a roadmap for scientific translation

Abstract: OBJECTIVECraniopharyngiomas are among the most challenging of intracranial tumors to manage because of their pattern of growth, associated morbidities, and high recurrence rate. Complete resection on initial encounter can be curative, but it may be impeded by the risks posed by the involved neurovascular structures. Recurrent craniopharyngiomas, in turn, are frequently refractory to additional surgery and adjuvant radiation or chemotherapy. Show more

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Cited by 30 publications
(27 citation statements)
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“…There are two subtypes of CP: aCP and papillary CP (pCP) [15,16]. The BRAF V600E mutation is characteristic of pCP, while most of aCP harbor a mutation of the β-catenin gene [15,17]. ACP has a high incidence in children and adolescents [15].…”
Section: Discussionmentioning
confidence: 99%
See 1 more Smart Citation
“…There are two subtypes of CP: aCP and papillary CP (pCP) [15,16]. The BRAF V600E mutation is characteristic of pCP, while most of aCP harbor a mutation of the β-catenin gene [15,17]. ACP has a high incidence in children and adolescents [15].…”
Section: Discussionmentioning
confidence: 99%
“…ACP has a high incidence in children and adolescents [15]. Recurrence in aCP is associated with increased vascular endothelial growth factor (VEGF) and fibroblast growth factor expression [17]. The aim of CP treatment is to maximize disease control while minimizing patient morbidity [18].…”
Section: Discussionmentioning
confidence: 99%
“…Targeted therapies directed against BRAF mutations have been successful in treating papillary craniopharyngioma in adults, but few agents have been tested against adamantinomatous craniopharyngioma, which is more common in children than in adults. 2 Hengartner et al offer an excellent review of the molecular biology of adamantinomatous craniopharyngiomas and present information about a number of molecular agents that can potentially target the pathways altered in these tumors. These agents targeting adamantinomatous craniopharyngioma could improve outcomes by both reducing the need for surgery/irradiation and decreasing the size of the tumor before surgery/irradiation.…”
mentioning
confidence: 99%
“…In contrast, PCPs are typically noncalcified, solid tumors that frequently harbor somatic BRAF V600E mutations that result in the activation of the MAPK signaling pathway [61]. To date, only few case reports demonstrated a sustained efficacy of the combination treatment with trametinib (MEK inhibitor) and dabrafenib (BRAF inhibitor) in patients with recurrent PCPs [62]. This significant progress has paved the way for a phase II clinical trial using a combination of vemurafenib and cometinib in patients with PCP (NCT-03224767).…”
Section: New Molecular Actors In the Pathophysiology Of Parasellar Lementioning
confidence: 99%