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  • Title: Endolymphatic sac tumour (ELST). Case report of a rare tumour of the temporal bone, presenting as a mass in a cerebello-pontine angle.
    Author: Czopek J, Waśko M, Adamek D.
    Journal: Folia Neuropathol; 2013; 51(2):164-8. PubMed ID: 23821389.
    Abstract:
    Endolymphatic sac tumour (ELST) is a primary low-grade, locally invasive adenocarcinoma of the endolymphatic sac, characterized by the proliferation of cuboidal cells forming a papillotubular pattern and colloid-filled cysts. Rare in the general population, it coincides significantly with the presence of von Hippel-Lindau disease. The natural history, mechanisms underlying the early symptoms, anatomical origin of ELST and optimal timing of their treatment are unknown. In this study, we report a Polish male patient with sporadic ELST (without a family history of VHL disease) along with a review of literature. The light microscopic and immunohistochemical features as well as clinical presentation were typical of ELST.
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