Predictive Factors for Pediatric Craniopharyngioma Recurrence: An Extensive Narrative Review

Abstract

Despite being classified as benign tumors, craniopharyngiomas (CPs) are associated with significant morbidity and mortality due to their location, growth pattern, and tendency to recur. Two types can be identified depending on age distribution, morphology, and growth pattern, adamantinomatous and papillary. The adamantinomatous CP is one of the most frequently encountered central nervous system tumors in childhood. Our aim was to review the relevant literature to identify clinical, morphological, and immunohistochemical prognostic factors that have been implicated in childhood-onset CP recurrence. Lack of radical surgical removal of the primary tumor by an experienced neurosurgical team and radiotherapy after a subtotal excision has been proven to significantly increase the recurrence rate of CP. Other risk factors that have been consistently recognized in the literature include younger age at diagnosis (especially <5 years), larger tumor size at presentation, cystic appearance, difficult tumor location, and tight adherence to surrounding structures, as well as the histological presence of whorl-like arrays. In addition, several other risk factors have been studied, albeit with conflicting results, especially in the pediatric population. Identifying risk factors for CP recurrence is of utmost importance for the successful management of these patients in order to ultimately ensure the best prognosis.

Hypogonadism as a consequence of craniopharyngioma in female patients: comparison of childhood and adult onset and effects of estrogen replacement therapy

“…In contrast, no major association between PR and ERα expression and clinical aggressiveness features was observed in CPs, according to detailed immunohistochemical investigation (Martínez-Ortega et al 2022). Estrogen therapy is not listed among the factors of the recurrence of the CP (Serbis et al 2023). Thus, we could assume that this therapy will be safe.…”

Evolving Aspects of Prognostic Factors for Pediatric Cancer

“…Craniopharyngiomas (CPs) are classified as non-malignant neoplasms but their location, growth pattern, and recurrence rate are associated with significant morbidity and mortality. The aim of the extensive narrative review by Serbis et al is to identify clinical, morphological, and immunohistochemical factors that are associated with the onset and, mainly, the recurrence of CP [11]. Molecular features such as BRAF gene mutations, altered p53 expression, increased Ki-67 expression, higher VEGF and HIF1a expression, and RARs have been extensively studied as potential predictive factors for CP relapse [11].…”

“…The aim of the extensive narrative review by Serbis et al is to identify clinical, morphological, and immunohistochemical factors that are associated with the onset and, mainly, the recurrence of CP [11]. Molecular features such as BRAF gene mutations, altered p53 expression, increased Ki-67 expression, higher VEGF and HIF1a expression, and RARs have been extensively studied as potential predictive factors for CP relapse [11]. Moreover, the decision of whether the surgical removal is followed by radiotherapy, age, adherence to surrounding tissues, histology, specific clinical findings, and molecular features remains to be validated through well-designed multicenter prospective studies, with the ultimate goal of developing targeted adjunct therapies [11].…”