Chinese expert consensus on the management of papillary craniopharyngioma(2025)
Chinese Anti-Cancer Association Neuro-Oncology Committee
Lin Zhixiong
Qi Songtao
Yang Xuejun
Abstract:Papillary craniopharyngioma(PCP)originates from the epithelial remnants of the craniopharyngeal duct.It is a tumor(CNS WHO Grade 1,ICD-O code 9352/1,with features of a tumor of borderline malignancy)driven by a somatic BRAF V600 E gene mutation and composed predominantly of well-differentiated non-keratinized squamous epithelium.The traditional treatment strategy for PCP primarily involves surgical resection,with postoperative residual tumor often managed with radiotherapy.The advent of novel therapies targeting the BRAF V600 E mutation has led to a paradigm shift in PCP management.Consequently,the Neuro-Oncology Committee of the Chinese Anti-Cancer Association convened experts from neurosurgery,pediatric neurosurgery,pediatrics,neuro-oncology radiotherapy,neuro-oncology chemotherapy,imaging,genetics,and endocrinology.This panel reached a consensus on the imaging features,diagnosis,pathological characteristics,molecular targeted therapy,radiotherapy,surgical therapy,endocrine therapy,and fundamental principles for managing papillary craniopharyngiomas.This consensus aims to further standardize the diagnosis and treatment of PCP,thereby improving patient quality of life and prognosis.
Keywords:Papillary craniopharyngiomaExpert consensusTargeted therapyRadiotherapyEndocrine therapy
Publication Date:2025-05-27
Online Publishing Date:2025-11-27(First online date of this platform, not the publication date of the document)
Pages:17( 5-21 )
