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首页> 外文期刊>Folia neuropathologica >Endolymphatic sac tumour (ELST). Case report of a rare tumour of the temporal bone, presenting as a mass in a cerebellopontine angle
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Endolymphatic sac tumour (ELST). Case report of a rare tumour of the temporal bone, presenting as a mass in a cerebellopontine angle

机译：内淋巴囊肿瘤（ELST）。颞骨罕见肿瘤的病例报告，小脑桥脑角呈肿块状

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摘要

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.
机译：内淋巴囊肿瘤（ELST）是内淋巴囊的一种原发性低度，局部浸润性腺癌，其特征在于长方体细胞的增殖形成了乳头状管型和胶体填充性囊肿。在普通人群中很少见，它与von Hippel-Lindau疾病的发生明显吻合。尚无自然史，早期症状的潜在机制，ELST的解剖学起源以及治疗的最佳时机。在这项研究中，我们报道了波兰男性散发性ELST患者（无VHL病家族史），并进行了文献综述。光学显微镜和免疫组化特征以及临床表现是ELST的典型特征。

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