Objective Endolymphatic sac tumors are rare neoplasms originating in the endolymphatic sac. Current literature is limited to case reports and small case series. The objective of this study was to systematically review the literature to better describe clinical presentation, treatment options, and outcomes in endolymphatic sac tumors. Data Source PubMed, Embase, and Cochrane Library. Review Methods A systematic review was performed following Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines describing human endolymphatic sac tumors. Risk of bias was assessed using a validated critical appraisal checklist for case series. Studies without inclusion of individual patient characteristics, corresponding treatments, and outcomes were excluded. Heterogeneity of data precluded meta-analysis. Results A total of 82 studies met inclusion criteria, and 253 discrete tumors were analyzed. A total of 23.4% of patients had von Hippel-Lindau disease. Von Hippel-Lindau–associated tumors affected females to males in a 2.4:1 ratio. Patients with von Hippel-Lindau disease displayed earlier average age at diagnosis compared to the sporadic cohort. Surgery was the primary treatment modality and was performed in 88.9% of cases. Adjuvant radiation therapy was employed in 18.7% of cases; 16.2% cases recurred, and 10.6% had progression of residual disease after treatment. Mean time to recurrence or progression was 53.1 ± 52.4 months with a range of 3 to 240 months. Conclusion Endolymphatic sac tumors require a high degree of suspicion for early diagnosis. Complete resection is the standard of care. No strong evidence supports routine use of adjuvant radiation therapy. Given the high rate of recurrence and wide-ranging time to recurrence, long-term follow-up is necessary.

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The petrous apex poses a challenge for surgical intervention due to poor access. As intraoperative image guidance and surgical instrumentation improve, newer endoscopic approaches are increasingly favored. This study aims to provide normative data on the anatomy of the lateral sphenoid sinus recess and petrous apex. These normative data could assist in determining the efficacy of a transnasal transsphenoidal approach to lesions of the anteroinferior petrous apex. This is a retrospective study investigating normative data on all maxillofacial computed tomography (CT) scans performed at a level I trauma center over a 6-month period. All appropriate images had the pneumatization pattern of the petrous apex and lateral recess of the sphenoid sinus reviewed by a single otologist and graded bilaterally. These were then analyzed in SPSS; Pearson correlation analyses and χ test were used. A total of 481 patients were identified, yielding a total of 962 temporal bones and sphenoid sinuses for analysis. Eighty-eight percent of sides analyzed had a nonpneumatized lateral recess. The petrous apex was nonpneumatized in 54% of sides analyzed. There was a correlation noted between the degree of pneumatization of the petrous apex and pneumatization of the lateral recess of the sphenoid. This study is the first to provide normative data comparing pneumatization of the petrous apex and sphenoid sinus. These data may support future work evaluating the utility of an endonasal approach to the petrous apex.