【双语病例】脑室内原发性中枢神经系统淋巴瘤

病史

A 65-year-old immunocompetent patient with headache of 3 months duration

65岁男性,头痛3个月。患者免疫功能正常。

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

图例Axial T2-FLAIR and T2WI (A, B) show two intraventricular lesions (arrows) with periventricular white matter edema, which are isointense to the gray matter. DWI (C) reveals restricted diffusion (similar to the white matter) within the tumor. Axial contrast-enhanced T1WI (D) demonstrates intense enhancement of the intraventricular lesions. MR spectroscopy (E) shows an elevated lipid peak, high Cho/Cr ratio, and decreased NAA.横断位T2-FLAIR及T2WI可见两处脑室内病灶,与脑灰质呈等信号,脑室周围白质水肿。

DWI示病灶轻度弥散受限,信号与脑白质相似。

增强后,横断位T1WI示脑室内病灶明显强化。

MRS示Lip峰升高,Cho/Cr比值升高,NAA峰降低。

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

【双语病例】脑室内原发性中枢神经系统淋巴瘤

Intraventricular Primary Central Nervous System Lymphoma

脑室内原发性中枢神经系统淋巴瘤

背景

  • Primary CNS lymphoma is a rare tumor accounting for 4% of all brain neoplasms.原发性中枢神经系统淋巴瘤是一种少见肿瘤,约占全部脑肿瘤的4%。
  • It is always the non-Hodgkin type, and the vast majority are B-cell lymphomas.几乎全部都是非霍奇金淋巴瘤,而且绝大部分为B细胞淋巴瘤。
  • Intraventricular primary CNS lymphoma is very rare, with few cases reported in the literature.脑室内原发性中枢神经系统淋巴瘤非常罕见,仅可见少量文献报道。
临床表现Diffuse headache over several weeks, weight loss, and gait disturbances弥漫性头痛,可持续数周,伴有体重减轻、步态不稳。

影像诊断要点

  • Lesions are hypo-to-isointense to the gray matter on T1WI and iso-to-hyperintense on T2WI.病灶于T1WI上,与灰质相比呈低或等信号;T2WI呈等或稍高信号。
  • In immunocompetent patients, there is almost always solid contrast enhancement.免疫功能正常的患者,病灶通常呈实性强化。
  • MR spectroscopy has demonstrated elevated lipid peaks combined with high Cho/Cr ratio and decreased peak of NAA.MRS一般表现为Lip峰升高,Cho/Cr比值升高,NAA峰降低。
  • A further workup with CT of the chest, abdomen, and pelvis can help confirm the absence of systemic disease involvement.患者需进一步行胸部、上腹部、盆腔CT,以排除全身其他部位受累。
鉴别诊断

  • Metastases 转移瘤
  • Choroid plexus carcinoma 脉络丛癌
  • Ependymoma 室管膜瘤
  • Meningioma 脑膜瘤
预后Primary intraventricular lymphomas seem to have a worse prognosis compared to the more common parenchymal type.与脑实质型的淋巴瘤相比,脑室内原发性淋巴瘤通常预后更差。

治疗Chemotherapy and radiotherapy.
化疗及放疗

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