eISSN: 1509-572x
ISSN: 1641-4640
Folia Neuropathologica
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vol. 51

Case report
Endolymphatic sac tumour (ELST). Case report of a rare tumour of the temporal bone, presenting as a mass in a cerebello-pontine angle

Jacek Czopek
Marcin Waśko
Dariusz Adamek

Folia Neuropathol 2013; 51 (2): 164-168
Online publish date: 2013/06/26
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Endolymphatic sac tumour (ELST) is a primary low-grade, locally invasive adenocarcinoma of the endolymphatic sac, charac-

terized 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.

endolymphatic sac tumour, ELST, temporal bone, von Hippel-Lindau disease

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