Abstract
Craniopharyngiomas are rare, benign primary brain tumors that arise from remnants of the craniopharyngeal duct epithelium within the sellar and suprasellar region. Despite their benign biology, they may cause significant morbidity, secondary to involvement of nearby eloquent neural structures, such as the pituitary gland, hypothalamus, and optic apparatus. Historically, aggressive surgical resection was the treatment goal to minimize risk of tumor recurrence via open transcranial midline, anterolateral, and lateral approaches, but could lead to clinical sequela of visual, endocrine, and hypothalamic dysfunction. However, recent advances in the endoscopic endonasal approach over the last decade have mostly supplanted transcranial surgery as the optimal surgical approach for these tumors. With viable options for adjuvant radiation therapy, targeted medical treatment, and alternative minimally invasive surgical approaches, the management paradigm for craniopharyngiomas has shifted from aggressive open resection to more minimally invasive but maximally safe resection, emphasizing quality of life issues, particularly in regards to visual, endocrine, and hypothalamic function. This review provides an update on current multi-modal approaches for craniopharyngiomas, highlighting the modern surgical treatment paradigm for this disease entity.
Highlights
Craniopharyngiomas are uncommon, benign central nervous system (CNS) tumors that arise from the remnants of the craniopharyngeal duct epithelium in the sellar and suprasellar region [1]
With the ability to expand the endoscopic endonasal approach beyond the transphenoidal approach into the transtuberculum and transplanum region, we have found that the majority of craniopharyngiomas are amenable to the endoscopic endonasal approach alone
Given the versatility of an expanded endoscopic endonasal approach, we resect the vast majority of craniopharyngiomas endoscopically as the initial approach and reserve the supraorbital craniotomy as a surgical adjunct for those with significant laterally located residual tumors, causing persistent compression of eloquent structures
Summary
Craniopharyngiomas are uncommon, benign central nervous system (CNS) tumors that arise from the remnants of the craniopharyngeal duct epithelium in the sellar and suprasellar region [1]. They represent approximately 2–5% of all primary intracranial tumors [2,3]. While adjuvant chemotherapy and/or radiation has limited efficacy, maximal surgical resection remains the mainstay of therapy. Current surgical treatment paradigms have evolved to tailor endoscopic endonasal, transcranial, or combined endoscopic endonasal and transcranial approaches to maximally resect craniopharyngiomas, based on individual tumor characteristics. This review discusses modern surgical approaches and adjunctive therapeutic modalities for the treatment of craniopharyngioma
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