The Challenging Management of Craniopharyngiomas in Adults: Time for a Reappraisal?

SIMPLE SUMMARY: Craniopharyngiomas (CPs) currently represent one of the most challenging diseases to deal with in the group of skull base tumors. Due to their location near, within, or surrounding the pituitary gland and stalk, CPs can be revealed by pituitary tumor syndrome and/or symptoms of hormonal deficiencies. Furthermore, surgery, which represents the first-line therapy, almost always results in hypopituitarism, diabetes insipidus and, in the case of hypothalamic involvement by the tumor, the occurrence of hypothalamic syndrome. The latter is characterized by intractable weight gain associated with severe morbid obesity, memory impairment, attention deficit, reduced impulse control and, eventually, increased risk of cardiovascular and metabolic disorders. Recent progress made in the understanding of the molecular pathways involved in CPs tumorigenesis paves the way for promising alternative therapeutic approaches and diagnostic procedures. Taken together, they lay the groundwork for new paradigms in the management of CPs in adults. ABSTRACT: Craniopharyngiomas (CPs) are rare tumors of the skull base, developing near the pituitary gland and hypothalamus and responsible for severe hormonal deficiencies and an overall increase in mortality rate. While surgery and radiotherapy represent the recommended first-line therapies for CPs, a new paradigm for treatment is currently emerging, as a consequence of accumulated knowledge concerning the molecular mechanisms involved in tumor growth, paving the way for anticipated use of targeted therapies. Significant clinical and basic research conducted in the field of CPs will undoubtedly constitute a real step forward for a better understanding of the behavior of these tumors and prevent associated complications. In this review, our aim is to summarize the multiple steps in the management of CPs in adults and emphasize the most recent studies that will contribute to advancing the diagnostic and therapeutic algorithms.