Infrasellar nasopharyngeal craniopharyngiomas: an individual participant data meta-analysis and review of the literature

Objective Craniopharyngiomas are benign tumors of the anterior skull base arising from epithelial remnants of Rathke’s pouch. They mainly occur in the suprasellar space, can be incredibly debilitating, and remain difficult to resect as they frequently involve critical neurovascular structures. Although it is embryologically possible for craniopharyngiomas to arise extracranially along the entire migrational path of Rathke’s pouch, these remain exceedingly rare, especially among adults, and can be mistaken for nasopharyngeal cancer. As such, minimal data exists evaluating the management and outcomes of such lesions. We evaluated our institutional experience with purely infrasellar nasopharyngeal craniopharyngiomas and obtained individual patient data reported in the contemporary literature to better characterize the demographics, presentation, surgical management, and long-term outcomes of these lesions. Methods A systematic review of the literature was performed to identify previously published cases of purely infrasellar nasopharyngeal craniopharyngioma in three electronic databases: MEDLINE (PubMed), Embase, Scopus. Search terminology included “infrasellar craniopharyngioma”, “nasopharyngeal craniopharyngioma”. Results Twenty-five total cases were identified and 72% presented with symptoms of nasal obstruction, epistaxis, or headache. 40% were resected via an endoscopic approach and 83.3% of all patients had a gross total resection with 60% having no recurrence at a median follow-up of 13 months. No patients had post-operative complications. Tumor location involving the cavernous sinus was associated with incomplete resection (100%) compared to those tumors not involving the cavernous sinus (87%), p = 0.033. Conclusion While uncommon, infrasellar nasopharyngeal craniopharyngiomas appear to have better perioperative and long-term surgical outcomes than their suprasellar counterparts.