Endolymphatic sac tumor is rare, locally aggressive and non-metastasizing neoplasm arising from the
endolymphatic sac of the petrous portion of the temporal bone. Most occur in adult and present with
ipsilateral hearing loss. They can be sporadic or associated with Von-Hippel-Lindau disease. Patient with
endolymphatic sac tumor should be screened for VHL disease. We report a case of 52-year-old female with
dizziness and headache. Histopathology was consistent with typical features of endolymphatic sac tumor.
This was confirmed by cytokeratin and EMA positivity and TTF-1 negativity. This case is presented for its
rarity with only few cases reported.
Endolymphatic Sac Tumor Associated with a Von Hippel-Lindau Disease Patient: An Immunohistochemical Study
