Clinicoradiologic characteristics of endolymphatic sac tumors.

PURPOSE: Endolymphatic sac tumor (ELST) is a rare, slow-growing, and low-grade malignant tumor arising from the endolymphatic sac in the posterior petrous bone. The purpose of this study is to describe the clinical and radiologic features, and investigate the clinicoradiologic correlation of ELST.

METHODS

We retrospectively reviewed the clinical, computed tomography (CT), magnetic resonance imaging (MRI), and pathologic findings of 14 patients with 15 ELSTs.

RESULTS

Patients comprised of eight women and six men with a mean age of 42.3 years at the time of diagnosis and 35.2 years at the time of initial symptoms. The mean interval between initial symptoms and diagnosis was 84.7 months. The most frequent cochleovestibular symptom was hearing loss in 14 patients (100%); other cochleovestibular symptoms were tinnitus in eight patients (57.1%), vertigo in three patients (21.4%), and aural fullness in three patients (21.4%). Ten patients (71.4%) presented with facial paralyses and five patients (14.3%) presented lower cranial nerve deficits. CT findings revealed spiculated, stippled, or reticular high density within the tumors. The lesions involved mastoid cells, vertical facial nerve canal, semicircular canal, cochlea, tympanum, jugular foramen, internal auditory canal, or petrous apex. On the available MRI, all the eight lesions showed patchy and/or speckled hyperintensity on unenhanced T1WI. Five lesions showed flow voids on T2WI and T1WI. Three lesions had blood fluid levels within cysts.

CONCLUSION

CT and MRI findings of ELSTs are associated with clinical features. Imaging tests should be performed to identify ELSTs early and ensure greater potential for hearing preservation in patients with cochleovestibular symptoms.