Abstract
Craniopharyngiomas (CPs) are slow growing, histologically benign intracranial tumors located in the sellar–suprasellar region. Although known to have low mortality, their location and relationship to the adjacent neural structures results in patients having significant neurologic, endocrine, and visual comorbidities. The invasive nature of this tumor makes complete resection a challenge and contributes to its recurrence. Additionally, these tumors are bimodally distributed, being treated with surgery, and are followed by other adjuncts, such as focused radiation therapy, e.g., Gamma knife. Advances in surgical techniques, imaging tools, and instrumentations have resulted in the evolution of surgery using endoscopic techniques, with residual components being treated by radiotherapy to target the residual tumor. Advances in molecular biology have elucidated the main pathways involved in tumor development and recurrence, but presently, no other treatments are offered to patients, besides surgery, radiation, and endocrine management, as the disease and tumor evolve. We review the contemporary management of these tumors, from the evolution of surgical treatments, utilizing standard open microscopic approaches to the more recent endoscopic surgery, and discuss the current recommendations for care of these patients. We discuss the developments in radiation therapy, such as radiosurgery, being used as treatment strategies for craniopharyngioma, highlighting their beneficial effects on tumor resections while decreasing the rates of adverse outcomes. We also outline the recent chemotherapy modalities, which help control tumor growth, and the immune landscape on craniopharyngiomas that allow the development of novel immunotherapies.
Highlights
Similar to SRS and Fractionated stereotactic radiotherapy (FSRT), studies have shown no variability in progression-free survival (PFS) or overall survival whether Intensity-modulated radiation therapy (IMRT) was delivered as adjuvant therapy or after remission in craniopharyngiomas
Proton beam therapy (PBT) seems extremely promising for the reduction of radiation toxicity in patients undergoing treatment for craniopharyngioma, the uncertainty in the clinical dose due to tissue heterogeneity demands further investigation to justify the greater cost of treatment [114]
Craniopharyngiomas are slow-growing tumors that need to be treated by a multidisciplinary team due to the long-term physical and psychological complications that can occur before and after their treatment
Summary
CPs account for 1% of all primary intracerebral tumors in adults and up to 15% in children. These tumors show an incidence of 0.18 cases per 100,000 inhabitants each year [1–3]. Despite the histologically non-malignant nature of CPs complete resection is challenging due to the proximity and invasiveness into adjacent neural tissue, enveloping major vasculature, and involving the hypothalamus and pituitary [2,5,6]. Survival at 5 years approximates 98%, there is a recurrence post-surgical comorbidities that impair daily functions [3].functions. That high rate ofand recurrence and post-surgical comorbidities that impair daily there are no distinctions in gender or race in the United. Tive risk for craniopharyngioma in black patients has been described [7]
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