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:
图B:
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。
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
- 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。
- 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）
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。
- 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 diseaseIgG-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