Surgical Treatment of Endolymphatic Sac Tumor With Adjunctive Stereotactic Radiation Therapy

—Case Report—

Abstract

A 44-year-old man presented with an endolymphatic sac tumor (ELST) associated with von Hippel-Lindau disease, which required four surgical procedures within 10 years. The earlier two surgeries resulted in only partial removal of the tumor because of vigorous intraoperative bleeding. Stereotactic radiation therapy was performed twice. The intraoperative bleeding was easy to control in the third operation, and ultimately the tumor was totally extirpated in the fourth operation. Histological examination of the tumor specimen harvested in the final surgery showed that the tumor cells had clearly decreased in number, and the interstitial tissue had become fibrous with organization of the tumor vessels, compared with the tumor specimen from the first surgery. Preoperative radiotherapy may be effective to reduce the devastating intraoperative bleeding of ELST.