Unusual Endolymphatic Sac Tumor: Case Report and Literature Review

Background: Endolymphatic sac tumors (ELSTs) are uncommon low-grade tumors with highly local aggressive features. These tumors could be associated with Von Hippel-Lindau disease or isolated. These tumors mostly present with audiovestibular and neurological symptoms. The definitive diagnosis can be achieved through histopathological examination of the post-surgical excised specimen. Case Presentation: We present a case of a female patient with a chief complaint of headache, unilateral hearing loss, and facial paralysis. Imaging studies demonstrated a mass in the left temporal bone. Intraoperatively, several hard, blood-stained fragments of bone and soft tissue. A complete microsurgical removal was achieved through a transmastoid-translabyrinthine approach with a preserved dura. Histopathological analysis confirmed the diagnosis of endolymphatic sac tumor. The patient was discharged with no new neurological deficit after the surgery with OPD follow up and referral for genetic studies. Conclusion: The reported case showed an unusual pre- and intraoperative presentation of endolymphatic sac tumor necessitating the need for early identification of such cases. Complete microsurgical removal remains the mainstay of treatment.