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Updated: Apr 21, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
Endolymphatic sac tumor as initial manifestation of von hippel-lindau syndrome
Pedro F Salazar1, Carol L Shields, Miguel A Materin
1From the Ocular Oncology Service, Wills Eye Institute, Thomas Jefferson University, Philadelphia, Pennsylvania.
Purpose:
To report endolymphatic sac tumor (ELST) as the initial finding in von Hippel-Lindau (VHL) syndrome.
Methods:
Case report.
Results:
A 4-year-old boy presented in February 2001 with right hearing loss and seventh nerve palsy from ELST. Genetic analysis showed a VHL mutation (gene 3p25-26). Systemic and ocular examination for VHL syndrome-related tumors was unrevealing. Four years later, multiple retinal hemangioblastoma (retinal capillary hemangioma) were detected in both eyes and successfully treated with argon laser photocoagulation.
Conclusion:
ELSTs can be the initial manifestation of VHL syndrome. Lifelong screening for related tumors, particularly retinal hemangioblastoma, is advised.
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