Demyelination preceding a diagnosis of central nervous system lymphoma

Demyelination preceding a diagnosis of central nervous system lymphoma
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DOI:
10.1016/j.jocn.2015.07.013
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发表时间:
2016-02-01
影响因子:
2
通讯作者:
Gaillard, Frank
Gaillard, Frank
中科院分区:
医学4区
文献类型:
--
作者:
Kalus, Sarah;Di Muzio, Bruno;Gaillard, Frank

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我们报告一位年轻免疫能力女性的原发性中枢神经系统淋巴瘤(PCNSL)合并脱髓鞘的病例。患者表现为右侧枕叶的扩张性强化病变,最初被归因于肿瘤性脱髓鞘,随后被证实为PCNSL。PCNSL是一种罕见的恶性肿瘤,尤其是在年轻的免疫功能正常的患者中,在MRI上典型的表现为均匀强化的孤立性肿块,好发于脑室周围和浅表部位,经常接触脑室和脑膜表面。肿瘤性脱髓鞘病变通常表现为大的白质病变,几乎没有肿块效应或血管源性水肿和“开环”强化,环的不完整部分位于病变的灰质一侧。PCNSL和肿瘤性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强调的那样,区分它们可能是具有挑战性的。我们讨论了如何结合临床和实验室结果应用常规和先进的MRI技术来获得准确的诊断,潜在地消除了对活检的需要,并促进了及时和适当的治疗。(C)2015爱思唯尔有限公司。保留所有权利。
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. (C) 2015 Elsevier Ltd. All rights reserved.