Korean J Otorhinolaryngol-Head Neck Surg.  2015 Oct;58(10):729-733. 10.3342/kjorl-hns.2015.58.10.729.

Hemangioma of Endolymphatic Sac in Von Hippel-Lindau Patient

• Yi YJ
• Kim Y
• Park SH
• Park MK

• KMID: 2100502
• DOI: http://doi.org/10.3342/kjorl-hns.2015.58.10.729

Abstract

The present study reports a rare case of capillary hemangioma of endolymphatic sac. A 23-year-old male who underwent von Hippel-Lindau disease presented with recurrent sudden sensorineural hearing loss. Magnetic resonance imaging revealed a heterogenous enhanced mass in the right endolymphatic sac, which was hyperintense on the enhanced T1-weighted images and inhomogenous on the T2-weighted images. Pre-operatively, this tumor was believed to be an endolymphatic sac tumor because of the history of von Hippel-Lindau disease. During the surgery, vascular tumor was removed by transmastoid approach. A histopathological examination indicated that the tumor was a capillary hemangioma. To the best of our knowledge, the present study is the second case of hemangioma in the endolymphatic sac and first case of von Hippel-Lindau disease.

Keyword

Endolymphatic sac tumor; Hemangioma; Sudden sensorineural hearing loss; Von Hippel-Lindau disease

MeSH Terms

Endolymphatic Sac*
Hearing Loss, Sensorineural
Hemangioma*
Hemangioma, Capillary
Humans
Magnetic Resonance Imaging
Male
von Hippel-Lindau Disease
Young Adult