Craniopharyngioma

Introduction Adamantinomatous craniopharyngioma (ACP) is a significant source of morbidity in the pediatric brain tumor population. It predominantly arises from the parasellar space. The tumors proximity to key vital structures often makes gross total surgical resection challenging and sometimes clinically inadvisable. Compounding the problem, adjunct therapies are not yet capable of providing a definitive cure. Recent preclinical research has made significant progress towards elucidating the mutagenic drivers of ACP, however much work remains to translate this into safe and effective treatments. Content Overview and Key Takeaways In the following chapter, we discuss the most up-to-date understanding of ACP biology and management. ACP is a histologically low grade tumor characterized by a mutation involving the beta-catenin protein, resulting in pathologic stability of the protein that impairs cell death or apoptosis. A subpopulation of tumor cells then enter a transcriptomic state characterized by cellular oncogenic senescence. It is unclear how this state leads to feed forward loops resulting in tumorigenesis. Despite its benign nature, the consistent anatomic origin of ACP in the sellar/suprasellar space, often abutting or involving the hypothalamus, the infundibulum, and the optic chiasm, contributes to multiple long-term complications such as endocrinopathies, obesity, vision loss, and obstructive hydrocephalus. Surgically, the current trend is towards conservative approaches with the goal of maximal safe resection without perturbing the hypothalamus. Adjunct radiotherapy is standard in cases of residual or recurrent disease. Close coordination with endocrinology colleagues is vital to appropriately care for these patients as they often suffer from lifelong endocrinopathies that remain a significant source of burden in this population.