A rare case of the supratentorial extraventricular anaplastic ependymoma. Case report and literature review

A. G. Gavrilov, D. Chelushkin, Ya. A. Latyshev, M. Ryzhova, T. Panina
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Abstract

Ependymoma is a brain tumor accounting for 1.9 % of all benign brain tumors and 3.1 % of glial tumors and 2–9 % of all neuroepithelial tumors. Approximately one third of intracranial ependymal tumors are supratentorial. They may be attached to the ependymal walls of the III ventricle and lateral ventricles or may be sited in the white matter without direct connection to the ventricular system pressing the adjacent cortex. In very rare cases ependymomas may lie cortically with blood supply from the dura – so-called «cortical» ependymomas.Posterior fossa ependymomas are more common in pediatric population with mean age of 6 years, whereas supratentorial ependymomas manifest in adults. In adults Grade III anaplastic ependymomas are most commonly seen.These tumors have no specific features and clinical manifestations of extraventricular anaplastic ependymomas may vary greatly depending on localization and size of the tumor.Supratentorial anaplastic ependymomas have no specific neuroimaging features either. Tumors are often hypo- and isointense in T1 and iso- or hyperintense in T2 sequence, Gd -inhancement is variable.Ependymomas are considered to be non-invasive and to have a strict border with brain tissue thus leading to clinical manifestations because of mass-effect.Surgical resection is the main treatment option for ependymoma. Patients with local Grade II tumor recurrence and patients with Grade III ependymomas should under go radiotherapy on the tumor r esection cavity.We present a rare case of a supratentorial extraventricular anaplastic ependymoma. A 21‑year-old female presented to the clinic with paresthesia and numbness in right hand, right half of lower lip and astereognosis. The magnetic resonance imaging of the brain showed a mass lesion in left fronto-parietal region. At 10.10.2017 surgery was performed and tumor was resected. Histological findings matched with features of anaplastic ependimoma, WHO Grade III. There was no tumor progression during 3 months after surgery.
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幕上室外间变性室管膜瘤1例。病例报告及文献复习
室管膜瘤是一种脑肿瘤,占所有良性脑肿瘤的1.9%,胶质肿瘤的3.1%,神经上皮肿瘤的2 - 9%。大约三分之一的颅内室管膜肿瘤位于幕上。它们可能附着在第三脑室和侧脑室的室管膜壁上,也可能位于白质中,与压迫邻近皮层的脑室系统没有直接联系。在非常罕见的病例中,室管膜瘤可能位于皮层,血液供应来自硬脑膜,即所谓的“皮质”室管膜瘤。后窝室管膜瘤多见于平均年龄为6岁的儿童,而幕上室管膜瘤见于成人。成人III级间变性室管膜瘤最常见。脑室外间变性室管膜瘤的临床表现可能因肿瘤的位置和大小而有很大差异。幕上间变性室管膜瘤也没有特定的神经影像学特征。肿瘤通常在T1序列呈低或等强度,在T2序列呈等或高强度,Gd增强是可变的。室管膜瘤被认为是非侵入性的,与脑组织有严格的边界,因此由于质量效应而导致临床表现。手术切除是室管膜瘤的主要治疗选择。局部II级肿瘤复发和III级室管膜瘤患者应在肿瘤切面腔行放射治疗。我们报告一例罕见的幕上室外间变性室管膜瘤。一名21岁女性以感觉异常、右手、下唇右半部分麻木及立体认知就诊。脑磁共振成像显示左侧额顶叶区肿块病变。于2017年10月10日行手术切除肿瘤。组织学表现符合间变性室管膜瘤的特征,WHO III级。术后3个月肿瘤无进展。
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