【双语病例】原发性硬脑膜淋巴瘤

DescriptionA
40-year-old man initially presented with gradually worsening headache. Two
months later, he presented with seizure. Body CT imaging and whole-body PET-CT
failed to reveal any other suspicious lesion.

40岁男性,头痛病逐渐加重。两个月后出现癫痫。体部CT及全身PET-CT未见明显异常。

图A:

【双语病例】原发性硬脑膜淋巴瘤

图B:

【双语病例】原发性硬脑膜淋巴瘤

LegendsA,
B:

Axial
T2WI (A, left) and contrast-enhanced T1WI (A, right) demonstrate enhancement of
the dura along the right temporal convexity (arrowhead) without parenchymal
signal change. Arrows indicate enhancing cortical veins.

横断位T2WI(A,左)及T1WI增强扫描(A,右)示右侧颞部大脑凸面硬脑膜强化,脑实质内未见明显异常信号。箭号所示为强化的皮层静脉。

【双语病例】原发性硬脑膜淋巴瘤

B,
top left; B, top right:

Axial
T2WI (B, top left) shows signal change in the right temporal lobe.
Contrast-enhanced T1WI (B, top right) reveals enhancement of the right
frontotemporal dura and leptomeninges, extending along the Sylvian fissure, with
infiltration of the right temporal lobe.

横断位T2WI(B,左上)示右侧颞叶异常信号,T1WI增强扫描(B,右上)右侧额颞部硬脑膜和软脑膜强化,突入外侧裂,并向右颞叶浸润。

B,
bottom left; B, bottom right:

Axial
DWI (B, bottom left) and ADC image (B, bottom right) demonstrate no associated
restricted diffusion.

横断位DWI(B,左下)和ADC图(B,右下)示病变无弥散受限。

Primary
Pachymeningeal (Dural) CNS Lymphoma

原发性硬脑膜淋巴瘤

Backgrounds

  • Primary
    pachymeningeal CNS lymphoma is rare and comprises 2.4% of primary CNS lymphoma
    (PCNSL).

    原发性硬脑膜淋巴瘤临床少见,约占全部原发性中枢神经系统淋巴瘤(PCNSL)的2.4%

  • Systemic
    lymphoma with secondary dural involvement must be excluded.

    需要排除全身性淋巴瘤继发的硬脑膜受累。

Clinical Information

  • More
    common in middle-aged females (PCNSL: older male)

    多见于中年女性。(PCNSL多见于老年男性)

  • Most
    frequent histology: Marginal zone B-cell lymphoma, low-grade indolent
    non-Hodgkin lymphoma (PCNSL: diffuse large B-cell lymphoma)

    常见的组织学类型:边缘区B细胞淋巴瘤,惰性非霍奇金淋巴瘤。(PCNSL多为弥漫性大B细胞淋巴瘤)

Key Diagnostic FeaturesUnifocal
or multifocal thickening/enhancement of the dura /- invasive features
(leptomeningeal enhancement, parenchymal infiltration). Solid component may show
restricted diffusion from hypercellularity.

硬脑膜单发或多灶性增厚,增强扫描可见强化,临近软脑膜或脑实质可正常或受侵。病变的实性部分由于细胞密度大而弥散受限。

DDx

  • Meningioma-en-plaque

    扁平型脑膜瘤

  • Dural
    metastases

    硬脑膜转移瘤

  • Tuberculosis

    结核

  • Sarcoidosis

    结节病(肉状瘤病)

  • IgG-4-related
    meningeal disease

    IgG-4相关硬脑膜病变

  • Idiopathic
    hypertrophic pachymeningitis

    特发性肥厚性硬脑膜炎

Rx

Systemic
or intrathecal chemotherapy and/or radiation

全身或鞘内化疗,或联合放疗

Leave a Comment

电子邮件地址不会被公开。 必填项已用*标注