腹部神经源性肿瘤

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1 腹部神经源性肿瘤神经髓鞘来源

2 简介腹部神经源性肿瘤范围很广, 主要包括神经节细胞来源 ( 神经节细胞瘤, 节细胞神经母细胞瘤, 神经母细胞瘤 ) 副神经节来源 ( 嗜铬细胞瘤, 副神经节细胞瘤 ) 神经髓鞘来源 ( 神经鞘瘤, 神经纤维瘤, 神经纤维瘤病, 恶性神经鞘膜瘤 )

3 简介神经髓鞘来源的肿瘤 90% 为良性, 多见于年轻和中年患者,10% 的病灶为多发男 : 女 (1:2) 患者通常没有明显临床症状, 病灶多为偶然发现神经鞘膜瘤较神经纤维瘤更多见

4 神经鞘膜瘤神经鞘膜瘤, 又称施旺细胞瘤, 属良性神经源性肿瘤, 起源于周围神经髓鞘肿瘤通常含包膜, 神经纤维位于病灶边缘神经鞘膜瘤几乎可发生在除头颈上下肢屈侧的人体任何部位人体深部的神经鞘膜瘤多见于后腹膜及后纵膈, 占后腹膜原发肿瘤的 6% 腹部的神经鞘膜瘤通常发生在脊柱旁, 邻近肾脏, 或盆腔内骶骨前区偶尔, 也可发生于腹壁, 膀胱, 或肠壁肿瘤直径多小于 5cm,, 但后腹膜的神经鞘膜瘤由于长期缓慢生长, 可比较大肿瘤的中心常由于血供不足而继发退行性改变 ( 如钙化囊变出血和玻璃样变 )

5 经组织学分析, 神经鞘膜瘤中通常包含两种细胞 ( Antoni A 型和 Antoni B 型 ) Antoni A 型 : 含丰富的梭形细胞, 常呈删状排列 ; Antoni B: : 肿瘤细胞被丰富的基质分隔

6 CT 及 MR 表现 CT 平扫 : 边界清晰的圆形或椭圆形肿块, 常伴有明显的囊变或钙化钙化可为斑点状斑片状或弧线形, 且钙化多位于外缘 CT 增强 : 肿瘤可均匀或不均匀强化, 其强化程度代表了肿瘤内含细胞数量的多少, 如 Antoni B 型肿瘤多为轻度强化肿瘤中央的低密度区也可能与囊变或出血等有关 MR:T1W 低信号,T2W 不均匀高信号, 增强后病灶外围多强化明显而中央强化常不明显

7 Multiple neurilemmomas in a 48-year-old woman. Coronal contrast-enhanced reformatted CT scan shows two lobulated soft-tissue-attenuation masses (arrows) in the left paravertebral space. The lower mass shows prominent cystic degeneration.

8 Retroperitoneal neurilemmoma in a 43-year-old woman. Contrast-enhanced CT scan shows a well-defined round mass in the left retroperitoneal space adjacent to the left kidney. The mass appears to have homogeneous low attenuation with a thick rim. Dense peripheral calcifications (arrows) are also seen in the periphery of the mass.

9 Neurilemmoma in a 35-year-old man. Contrast-enhanced CT scan shows a tumor with poor contrast enhancement (arrow) in the anterior abdominal wall. The tumor causes splaying of the left rectus abdominis muscle.

Retroperitoneal neurilemmoma in a 38-year-old woman. (a) Contrast-enhanced CT scan shows a well defined low-attenuation mass adjacent to the right kidney.

10 Retroperitoneal neurilemmoma in a 38-year-old woman. (a) Contrast-enhanced CT scan shows a well defined low-attenuation mass adjacent to the right kidney. (b) Axial T1- weighted MR image shows a homogeneous low-signal-intensity intensity tumor (arrow). (c) On an axial turbo spin-echo T2-weighted MR image, the tumor has heterogeneous high signal intensity and is located posterior to the inferior vena cava (V). (d) Axial contrast-enhanced T1-weighted MR image shows the mass with inhomogeneous contrast enhancement (arrow), although it looked like a cystic tumor at CT (cf a).

11 神经纤维瘤 / 神经纤维瘤病神经纤维瘤可以为一单发的肿瘤或神经纤维瘤病的一部分其中,90% 为单发神经纤维瘤,10% 见于 von Recklinghausen 病男性发病率高于女性,20~40 岁好发与神经鞘膜瘤比较, 神经纤维瘤更易恶变 ( 尤其是神经纤维瘤病 ) 神经纤维瘤同样起源于神经髓鞘, 组织学分析中可见增厚呈波浪状的胶原纤维散布在增生的神经鞘膜细胞中, 可见不同程度的粘液样变性与神经鞘膜瘤不同, 神经纤维瘤没有包膜, 也无法明确区分为 Antoni A 或 B 型

12 神经纤维瘤病灶内可见神经纤维穿过, 这与神经鞘膜瘤非常不同此外, 神经纤维瘤大体上为质地坚韧的实性肿块 ; 镜下, 退行性变, 细胞数减少及黄色瘤样物质少见

14 CT 及 MR 表现 CT 扫描中, 神经纤维瘤表现为密度均匀边缘光滑的类圆形软组织肿块, 平扫时 CT 值约 HU,, 增强后强化多较均匀,CT 值约 HU Kumar et al 提出, 神经纤维瘤中的低密度区很可能与富含脂质的 Schwann 细胞脂肪细胞及嵌入的邻近脂肪组织有关 ; 而高密度区应与致密的胶原组织有关神经纤维瘤可由于粘液样变性而出现大小不一的多发囊性区 T1W 中, 病灶中央信号高于外周 ;T2W 中, 外周信号则高于中央 ; 提示病灶中央为神经组织, 外周为粘液样变区

15 神经纤维瘤病为一组由异种基因引发的遗传性疾病, 可分别两大类型 :NF-1 和 NF-2 NF-1 主要累及皮肤和周围神经系统, 可见 cafe - au-lait 斑和沿周围神经走向分布的皮下结节孤立神经纤维瘤患者中, 30% 实为 NF-1 患者, 而所有的神经纤维瘤病或丛状神经纤维瘤病患者都属 NF-1

16 在腹盆腔,NF-1 可累及腹腔内数量众多的神经节及支配腹盆腔脏器的体神经或自主神经病灶可位于后腹膜系膜和脊柱旁 CT 中, 来源于系膜或后腹膜的神经纤维瘤常与肾上腺肿瘤发生混淆盆腔内的丛状神经纤维瘤病多表现为较大的广泛浸润性的骶前区 / 臀区的肿块 NF-2 的特点为双耳前庭的施旺细胞瘤和多发的脑 ( 脊 ) 膜瘤, 几乎不累及周围神经系统

(b) On a sagittal contrastenhanced T1-weighted MR image, the tumor has heterogeneous contrast enhancement (U) anteriorly and the rectum.

17 Extraperitoneal pelvic neurofibroma in a 59-year-old man. (a) Sagittal T2- weighted MR image shows a well-defined round tumor in the pelvic cavity with mixed low and high signal intensity due to myxoid degeneration. (b) On a sagittal contrastenhanced T1-weighted MR image, the tumor has heterogeneous contrast enhancement (U) anteriorly and the rectum. (15) Neurofibroma originating from the urinary bladder in a 55-year-old man. Contrast-enhanced CT scan shows a well-enhanced enhanced solid mass that abuts the left lateral wall of the urinary bladder (U) and has marked internal heterogeneity due to myxoid degeneration. Occasionally, neurogenic tumors can involve the bladder.

18 Neurofibroma in the left iliac fossa in a 34-year-old woman. Contrast-enhanced CT scan shows a mass with a target-like enhancement pattern. The central portion of the mass (nerve tissue) is more hyperattenuating than the periphery (myxoid degeneration).

19 Plexiform neurofibromatosis in a 17-year-old girl. Contrast-enhanced CT scan demonstrates multiple low-attenuation nodular lesions (arrows) along the right paravertebral region. Note the scalloping of the adjacent vertebral body.

PowerPoint Presentation

PowerPoint Presentation 肠系膜脂膜炎的影像诊断张薇 2010-6-21 肠系膜主要由脂肪及穿行于其中的供应肠道的动静脉和淋巴管构成 Gudinchet 等早在 1927 年提出回缩性系膜炎 (Retractile mesenteritis,rm) ; 1947 年 Pemberton 等描述肠系膜脂肪营养