Contemporary Biological Insights and Clinical Management of Craniopharyngioma

Abstract Craniopharyngiomas (CPs) are clinically aggressive tumors because of their invasive behavior and recalcitrant tendency to recur after therapy. There are 2 types based on their distinct histology and molecular features: the papillary craniopharyngioma (PCP), which is associated with BRAF-V60...

Full description

Saved in:
Bibliographic Details
Published in:Endocrine reviews 2023-06, Vol.44 (3), p.518-538
Main Authors: Apps, John Richard, Muller, Hermann Lothar, Hankinson, Todd Cameron, Yock, Torunn Ingrid, Martinez-Barbera, Juan Pedro
Format: Article
Language:English
Subjects:
Brain
Clinical trials
Comorbidity
Craniopharyngioma - diagnosis
Craniopharyngioma - genetics
Craniopharyngioma - therapy
Development and progression
Gene mutations
Health services
Histology
Humans
Hypothalamus
Intracranial pressure
Irradiation
Mutation
Neoplasia
Pathogenesis
Patients
Pituitary
Pituitary gland
Pituitary gland tumors
Pituitary Neoplasms - diagnosis
Pituitary Neoplasms - genetics
Pituitary Neoplasms - therapy
Quality of Life
Radiation therapy
Radiotherapy
Refilling
Tumors
Vismodegib
Visual pathways
β-Catenin
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Abstract Craniopharyngiomas (CPs) are clinically aggressive tumors because of their invasive behavior and recalcitrant tendency to recur after therapy. There are 2 types based on their distinct histology and molecular features: the papillary craniopharyngioma (PCP), which is associated with BRAF-V600E mutations and the adamantinomatous craniopharyngioma (ACP), characterized by mutations in CTNNB1 (encoding β-catenin). Patients with craniopharyngioma show symptoms linked to the location of the tumor close to the optic pathways, hypothalamus, and pituitary gland, such as increased intracranial pressure, endocrine deficiencies, and visual defects. Treatment is not specific and mostly noncurative, and frequently includes surgery, which may achieve gross total or partial resection, followed by radiotherapy. In cystic tumors, frequent drainage is often required and intracystic instillation of drugs has been used to help manage cyst refilling. More recently targeted therapies have been used, particularly in PCP, but also now in ACP and clinical trials are underway or in development. Although patient survival is high, the consequences of the tumor and its treatment can lead to severe comorbidities resulting in poor quality of life, in particular for those patients who bear tumors with hypothalamic involvement. Accordingly, in these patients at risk for the development of a hypothalamic syndrome, hypothalamus-sparing treatment strategies such as limited resection followed by irradiation are recommended. In this review, we provide an update on various aspects of CP, with emphasis on recent advances in the understanding of tumor pathogenesis, clinical consequences, management, and therapies. Graphical Abstract
ISSN:0163-769X
1945-7189
DOI:10.1210/endrev/bnac035