Rare sporadic endolymphatic sac tumors: A case report and literature review

Abstract

Endolymphatic sac tumor (ELST) is a rare pathology, with fewer than 300 cases reported worldwide. These tumors are characterized by slow but invasive growth and malignancy, primarily manifesting as local invasion, while distant metastases are rare. We report a sporadic case of a 36-year-old woman with a histologically verified ELST. There was no previous medical history or clinical data suggesting Von Hippel-Lindau disease, nor family members with the condition. The patient experienced a gradual onset of symptoms over seven years, reflecting the tumor's indolent nature. Diagnostic workup included imaging and histopathological evaluation. Surgical resection was performed, and the diagnosis was confirmed postoperatively. We discuss the diagnostic challenges, therapeutic approach, and follow-up of patients with ELST in the context of current literature and management recommendations. This case highlights the importance of early recognition and individualized care in patients with ELST, even without associated syndromic features.