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中枢神经系统淋巴瘤诊断前的脱髓鞘病变。

Demyelination preceding a diagnosis of central nervous system lymphoma.

作者信息

Kalus Sarah, Di Muzio Bruno, Gaillard Frank

机构信息

Department of Radiology, 1st Floor, 1B Building, The Royal Melbourne Hospital, Grattan Street, Parkville, VIC 3050, Australia.

Department of Radiology, 1st Floor, 1B Building, The Royal Melbourne Hospital, Grattan Street, Parkville, VIC 3050, Australia.

出版信息

J Clin Neurosci. 2016 Feb;24:146-8. doi: 10.1016/j.jocn.2015.07.013. Epub 2015 Oct 9.

Abstract

We present a case of primary central nervous system lymphoma (PCNSL) co-existing with demyelination in a young immunocompetent woman. The patient presented with an expansile, enhancing lesion in the right occipital lobe which was initially attributed to tumefactive demyelination and subsequently proven to be PCNSL. PCNSL is an uncommon malignancy, particularly in young immunocompetent patients, and on MRI classically manifests as a homogeneously enhancing solitary mass with a predilection for periventricular and superficial locations, often contacting ventricular and meningeal surfaces. Tumefactive demyelinating lesions typically present as large white matter lesions with little mass effect or vasogenic oedema and "open-ring" enhancement, with the incomplete portion of the ring on the grey matter side of the lesion. PCNSL and tumefactive demyelinating lesions share some radiological features and thus, as our case report highlights, differentiating between them can be challenging. We discuss how the application of conventional and advanced MRI techniques combined with clinical and laboratory findings can lead to a precise diagnosis, potentially obviating the need for biopsy and facilitating prompt and appropriate treatment.

摘要

我们报告一例年轻免疫功能正常女性原发性中枢神经系统淋巴瘤(PCNSL)与脱髓鞘并存的病例。患者右侧枕叶出现一个有强化的膨胀性病变,最初被认为是瘤样脱髓鞘,后来证实为PCNSL。PCNSL是一种罕见的恶性肿瘤,尤其是在年轻免疫功能正常的患者中,在MRI上通常表现为均匀强化的孤立肿块,倾向于位于脑室周围和浅表部位,常与脑室和脑膜表面接触。瘤样脱髓鞘病变通常表现为大的白质病变,几乎没有占位效应或血管源性水肿,呈“开环”强化,环的不完整部分位于病变的灰质侧。PCNSL和瘤样脱髓鞘病变有一些共同的放射学特征,因此,正如我们的病例报告所强调的,区分它们可能具有挑战性。我们讨论了如何应用传统和先进的MRI技术,结合临床和实验室检查结果,从而做出准确诊断,可能避免活检的需要,并有助于及时进行适当治疗。

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