Head & Neck, Endolymphatic sac tumour [LMP62694]

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Image Details
Diagnostic Modality: 
Whole Slide Image
Disease Category: 
Neoplasia
Diagnosis: 
Endolymphatic sac tumour
Species: 
Human
Organ System: 
Head and Neck
Gender: 
Female
Age: 
48 years
Diagnosis Code: 
ICD-10: C41.0 | ICD-O: 8140/3
Slide Contributor Information
Gao, A., Kiehl, TR.

Keywords

Von Hippel-Lindau, ELST, aggressive papillary tumour

Clinical History

48-year-old woman with left petrous temporal bone tumour.

Clinical Discussion

Endolymphatic sac tumour is an epithelial tumour arising within the endolymphatic sac/duct and is highly associated with von Hippel-Lindau syndrome. It generally presents in adulthood with unilateral hearing loss. Microscopically, the tumour often demonstrates bone invasion and shows a papillary architecture with a single layer of low cuboidal to columnar epithelial cells. The cytoplasm is clear to granular eosinophilic; there are indistinct cell borders; nuclei are small, round, and hyperchromatic. Prognosis is generally good although complete surgical resection is often difficult to achieve.