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Ear Endolymphatic sac tumour in VHL syndrome: The middle ear endolymphatic sac tumour is a well-recognized tumour associated with von Hippel Lindau syndrome in over a third of the cases and it is the initial manifestation of VHL in > 30% of individuals with these lesions. The neoplastic cells show a low cuboidal to columnar cell appearance, lining papillary projections and dilated cystic spaces. Secretory colloid-like material is frequently seen in cystic spaces
