Current Advances in Papillary Craniopharyngioma: State-Of-The-Art Therapies and Overview of the Literature

Abstract

Craniopharyngiomas are commonly classified as low-grade tumors, although they may harbor a malignant behavior due to their high rate of recurrence and long-term morbidity. Craniopharyngiomas are classically distinguished into two histological types (adamantinomatous and papillary), which have been recently considered by the WHO classification of CNS tumors as two independent entities, due to different epidemiological, radiological, histopathological, and genetic patterns. With regard to papillary craniopharyngioma, a BRAF V600 mutation is detected in 95% of cases. This genetic feature is opening new frontiers in the treatment of these tumors using an adjuvant or, in selected cases, a neo-adjuvant approach. In this article, we present an overview of the more recent literature, focusing on the specificities and the role of oncological treatment in the management of papillary craniopharyngiomas. Based on our research and experience, we strongly suggest a multimodal approach combining clinical, endocrinological, radiological, histological, and oncological findings in both preoperative workup and postoperative follow up to define a roadmap integrating every aspect of this challenging condition.

Advancing Craniopharyngioma Management: A Systematic Review of Current Targeted Therapies and Future Perspectives

“…This is not to be confused with information sources (see first footnote). ‡ The frameworks by Arksey and O'Malley (6) and Levac and colleagues (7) and the JBI guidance (4,5) refer to the process of data extraction in a scoping review as data charting. § The process of systematically examining research evidence to assess its validity, results, and relevance before using it to inform a decision.…”

“…This precision allows for the reduction in treatmentrelated complications and the preservation of critical neurological and endocrine functions. Additionally, the observed responses, including substantial reductions in tumor size and improved clinical outcomes, highlight the promise of targeted therapies in achieving more favorable long-term results [6]. The ability of targeted therapies to address the underlying genetic alterations in craniopharyngiomas suggests a personalized approach to treatment, tailoring interventions based on the unique molecular profile of each patient's tumor.…”

“…The ability of targeted therapies to address the underlying genetic alterations in craniopharyngiomas suggests a personalized approach to treatment, tailoring interventions based on the unique molecular profile of each patient's tumor. Moreover, the documented responses in cases refractory to traditional therapies suggest the potential for targeted therapies to serve as salvage options, providing hope for patients with limited alternative treatment options [1,3,6].…”

Adamantinomatous craniopharyngioma: evolution in the management

“…Further, potential targets could come from the analysis of the clonal mutations that drive the oncogenesis of craniopharyngioma [ 186 , 187 ]. AC is characterized by alterations in the Wnt/b-catenin pathway, mainly involving the central regulatory gene CTNNB1, while papillary craniopharyngioma is mostly driven by the V600E mutation in the BRAF gene, which activates the mitogen-activated protein kinase (MAPK) signaling pathway [ 186 – 189 ].…”