Endolymphatic sac tumor in a patient with von Hippel-Lindau disease: MR imaging findings
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Updated: Aug 10, 2026
Surgical Treatment of an Endolymphatic Sac Tumor
Published on: May 26, 2023
1Department of Radiology, Dört D Millet Hospital, Tekirdağ, Turkey. okilickesmez@yahoo.com
Abstract:
A 29-year-old male patient with von Hippel-Lindau disease who had an operation for cerebellar hemangioblastoma six years ago is presented. The patient had a four-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by MR imaging, was almost completely cystic in appearance. Pathological examination after removal of the mass confirmed a papillary endolymphatic sac tumor.
Insights
Von Hippel-Lindau disease can present with rare temporal bone tumors. This case highlights a papillary endolymphatic sac tumor in a patient with this genetic disorder.
Area of Science:
- Neuro-oncology
- Otolaryngology
- Genetics
Background:
- Von Hippel-Lindau (VHL) disease is a rare genetic disorder predisposing individuals to various tumors.
- Cerebellar hemangioblastomas are a common manifestation, often requiring surgical intervention.
Observation:
- A 29-year-old male with a history of VHL and prior cerebellar surgery presented with a 4-year history of a slow-growing temporal bone lesion.
- The lesion involved the cerebellopontine angle and was characterized on MR imaging as predominantly cystic.
- The mass was locally invasive and vascularized.
Findings:
- Surgical resection of the temporal bone mass was performed.
- Pathological examination confirmed the diagnosis of a papillary endolymphatic sac tumor.
Implications:
- This case expands the known spectrum of tumors associated with Von Hippel-Lindau disease.
- Highlights the importance of considering rare tumor types in VHL patients, even in unusual locations.
- Suggests the need for comprehensive diagnostic workups for complex cranial base lesions in VHL patients.
