A rare clinical case of a labyrinthine lesion: an endolymphatic sac tumor

Abstract

Endolymphatic sac tumors are rare neoplasms originating from the endolymphatic epithelium of the intraosseous part of the endolymphatic duct and sac, typically characterized by slow growth and local destructive changes. We present a case of an endolymphatic sac tumor in a 28-year-old man who consulted an otoneurologist with complaints of progressive hearing loss and dizziness attacks and was referred for a CT scan of the temporal bones and MRI of the inner ear with delayed contrast enhancement. The data of otoneurological and radiological examinations before and after surgical treatment and the results of surgical treatment with histological verification of the tumor are presented. The objective of this case presentation is to demonstrate the usefulness of CT and MRI methods in detecting an endolymphatic sac tumor, to compare the results of otoneurological and radiological examinations and intraoperative and histological data followed by surgery. Radiological examination revealed a neoplasm of the right temporal bone with typical imaging characteristics of endolymphatic sac tumor. Surgery was performed, the mass was removed completely, and the endolymphatic sac tumor was histologically verified. Radiologic presentation includes a typically located area of bone destruction in the petrous part of the temporal bone in the area of the vestibular aqueduct, possibly with the spread of the tumor into the cerebellopontine angle, with a heterogeneous structure, with uneven contrast enhancement. Due to the rarity of this pathology, diagnosis in the early stages is often difficult, and for the correct interpretation of radiologic findings in patients with hearing loss and dizziness, knowledge of the normal anatomy of the temporal bone and awareness of rare variants of its lesions are of key importance. Timely examination of patients with suspected temporal bone lesions and correct diagnosis are the key to successful treatment and maintaining the quality of life in this group of patients.