Endolymphatic Sac Tumors: Case Series and Literature Review

Introduction: The endolymphatic sac tumor (ESC) consists of an embryological neoplasm in the neuroectodermal tissue, rare, with less than 300 cases reported in the literature, indolent growth, benign but locally aggressive. This tumor develops sporadically or as part of the Von Hippel-Lindau autosomal dominant disease (VHL).

Materials and Method: This is a retrospective study with the analysis of seven patients who underwent surgical procedure for resection of endolymphatic sac tumors by a single team in the past 30 years and review of literature.

Results: Seven patients, 5 women and 2 men with mean age between 36 and 79 years (average: 55.8). Hearing loss was the most common symptom, affecting 100% of patients, with 5 presenting with anacusia and 2 presenting with hypoacusia. Vertigo was another common symptom (42.85%), followed by pulsatile tinnitus (28.57%). Facial nerve paralysis was present in all patients at the time of diagnosis, presenting House-Brackmann (HB) II to VI. Three patients had associated with von Hipple–Lindau syndrome. Access to the cranial base was performed in seven patients and complete resection was performed in five of them.

Conclusion: Recent diagnosis and early approach lead to better prognoses. Radiotherapy can be considered only in patients with recurrent residual lesions.

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Artikel online veröffentlicht:
07. Februar 2025