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斜坡软骨肉瘤伴轴内囊性髓质病变,对类固醇有反应。

Clival Chondrosarcoma Associated With an Intra-Axial Cystic Medullary Lesion Responsive to Steroids.

作者信息

Walter Johannes, Kapitza Sandra, Krayenbühl Niklaus, Tarnutzer Alexander A

机构信息

Department of Neurosurgery, University Hospital Zurich, Zurich, Switzerland.

Faculty of Medicine, University of Zurich, Zurich, Switzerland.

出版信息

Front Neurol. 2018 Jun 26;9:502. doi: 10.3389/fneur.2018.00502. eCollection 2018.

Abstract

Here we present a 75-year-old patient who was admitted with acute-onset right-sided hemiparesis, dysphagia, dysarthria and nystagmus. Repeated MRI scans showed two lesions with contact to one another: one solid stationary extra-axial lesion at the caudal part of the clivus and a rapidly growing intra-axial cystic lesion at the level of the medulla oblongata. Biopsy of the solid lesion demonstrated a low-grade chondrosarcoma, while no tissue sample of the cystic lesion could be retrieved. After initiation of dexamethasone therapy the cystic lesion markedly regressed. A literature search on published cases with the same combination of a stationary solid extra-axial mass at the caudal part of the clivus and a growing intra-axial cystic mass in the medulla oblongata was negative, indicating that the case described here is both unique and novel. Considering the rapid progression of symptoms and growth on MR-imaging in combination with the marked response to steroids, an inflammatory response linked to the chondrosarcoma is most likely. At the same time other possible explanations as a second neoplasm, an abscess or an ischemic lesion seem unlikely. This case underlines an unusual complication of a rare brainstem tumor and outlines both the differential diagnosis and potential treatment options. For such cystic lesions in combination with chondrosarcoma, a treatment course with steroids should be considered along with surgical exploration necessary to obtain the diagnosis and for potential reduction of mass-effect on the medulla oblongata.

摘要

我们在此介绍一位75岁的患者,该患者因急性起病的右侧偏瘫、吞咽困难、构音障碍和眼球震颤入院。多次MRI扫描显示两个相互接触的病灶:一个位于斜坡尾部的实性、静止的轴外病灶,以及一个位于延髓水平快速生长的轴内囊性病灶。实性病灶活检显示为低级别软骨肉瘤,而囊性病灶未获取到组织样本。地塞米松治疗开始后,囊性病灶明显缩小。对已发表的病例进行文献检索,结果显示没有与斜坡尾部的静止实性轴外肿块和延髓内生长的轴内囊性肿块同时出现的相同组合的病例,表明此处描述的病例既独特又新颖。考虑到症状的快速进展和MR成像上的生长情况,以及对类固醇的显著反应,最有可能是与软骨肉瘤相关的炎症反应。同时,作为第二种肿瘤、脓肿或缺血性病变的其他可能解释似乎不太可能。该病例强调了一种罕见脑干肿瘤的不寻常并发症,并概述了鉴别诊断和潜在的治疗选择。对于这种与软骨肉瘤相关的囊性病灶,应考虑使用类固醇进行治疗,并进行手术探查以明确诊断并可能减轻对延髓的占位效应。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9eed/6028611/8e63d4121178/fneur-09-00502-g0001.jpg

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