Castleman病的影像学诊断与鉴别诊断

2016-09-20 放射沙龙 放射沙龙

作者:toroto版权申明:谢绝转载出品方:深圳市放射沙龙文化传媒有限公司投稿邮箱:2519330936@q

简述

Castleman 病(Castleman disease,CD)又称血管滤泡性淋巴结增生或巨淋巴结增生症,最早由 Benjamin Castleman 于1954年报道。该病可表现为单发或多发,且可发生于全身各处的淋巴组织,以颈部、纵隔、肺门及腹部等淋巴结较多的部位较为常见。CD的病因和发病机制尚不明确,确诊主要依靠病理活检,治疗为以手术切除为主的综合治疗。

Castleman 病是一组临床和病理与淋巴瘤相似的少见的原因不明的淋巴增殖性疾病,病理特征为:明显的淋巴滤泡、血管及浆细胞呈不同程度的增生。临床上CD分为局灶型和多中心型,病理组织学分为透明血管型、浆细胞型和混合型。

本病最突出的临床表现是无痛性淋巴结肿大,可发生于身体的任何部位,大多数病例表现为局限性淋巴结病变,最常侵犯纵膈淋巴结,颈部、后腹壁、腋窝及盆腔等部位淋巴结也常被侵犯,有时本病还可出现于肌肉、肺部、眼眶等没有淋巴结的部位。

CD的影像学表现

CD的影像学表现与病理分型密切相关。透明血管型多表现为局限型。表现为单发软组织肿块,密度多较均匀,强化特点为早期均匀明显强化,CT值接近主动脉,延迟期仍持续强化,与透明血管型有较多的供养血管,加之病灶血管增生伴毛细血管异常增生和扩张有关。病灶内极少伴有出血和坏死是局限型CD的另一个特征,与CD血供丰富及淋巴滤泡本身不易坏死有关。病变内部的弧形、分枝状或簇状钙化也是CD的特点之一。

浆细胞型CD多为弥漫型,由于血管成分较少,其强化程度不如透明血管型,一般呈轻中度强化。弥漫型CD影像学上缺乏特征,容易引起误诊。

鉴别诊断

局限型CD需与富血供肿瘤相鉴别,如胸腺瘤、嗜铬细胞瘤、神经源性肿瘤等,与大动脉相似的强化方式,内少见囊变坏死及弧形、分枝状钙化可作为局限型CD与其他富血供肿瘤的鉴别特点

弥漫型CD需与淋巴瘤、结节病、淋巴结核鉴别。与淋巴瘤的鉴别最为困难,两者均可表现为全身多发淋巴结肿大和肝脾肿大,淋巴瘤可出现血管包埋征但血管形态正常可作为两者鉴别诊断的重要特点。

病例分享

一、腹膜后Castleman 病,平扫呈类圆形等密度灶,边界清,动脉期明显强化,静脉其及延迟期持续强化。

二、颈部Castleman 病

 

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    2021-11-10 fengyi812
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    2021-11-10 yaanren

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