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PHILIPS每日一例:多肺叶密集结节伴大量咳血的罕见病例报告

2022-09-17 放射沙龙 放射沙龙

肺 EHE 在无恶性肿瘤风险的患者通常表现为肺内多发结节。女性较常见,多为中年患者。在肺 EHE 诊断初患者多无症状,诊断多为偶然。在有症状患者,可常见呼吸困难、咳嗽、胸痛、咯血及体重减轻等。

病史回顾:白人女性,40 岁“轻微咳嗽、发热,伴咯出少量淡红色鲜血加重 1 周”,否认呼吸困难、咳痰、鼻衄、呕血及体重减轻。有吸烟史, 20 年前因慢性胰腺炎、慢性疼痛予空肠导管鼻饲及慢性四级镇痛药。否认恶性肿瘤及肺部疾病。

体格检查:双肺呼吸音低,右肺底部可及弥漫性湿罗音,余无殊。

诊断过程:患者有轻度的白细胞增多(WBC 14000/μL)中性粒细胞为主,凝集实验正常。胸片示:右肺下叶实体肿物占位(图 1)

图 1. 起初的胸片显示右肺下叶实体肿物,附见可见心影边界增大,导管头位于右心房内

患者起病时诊断为社区获得性肺炎并予抗生素治疗,症状加重并伴有持续性大量咯血、呼吸困难及血氧饱和度不足。肺部 CT 示:双肺多发弥漫性透明结节样影(图 2)。

图 2.A, B, 胸部 CT 示:多发性弥漫性肿块样透明结节,最大者位于右肺下叶 4.0*2.7cm,A. 横断面,B. 冠状面. 右肺上叶、左肺上叶及左肺下叶可见多发透明样结节,有些结节具胸膜下成分,少量的右肺胸膜渗出。另外,胸内气管旁及双侧肺门淋巴结肿大

支气管镜可见右肺中叶持续性渗血,予选择性右支气管动脉栓塞;支气管肺泡灌洗液及支气管镜活检标本分析均未发现感染、恶性肿瘤等病因。由于持续性咯血,予胸腔镜切除右中肺叶。术后病理示:(右肺结节)大量出血、坏死及广泛性圆形、卵圆形上皮细胞增殖。免疫组化染色示:ERG、CD31、Fli-1 均阳性,胸内淋巴结及心包结节也发现这些改变。

诊断:肺上皮样血管内皮瘤(Pulmonary epithelioid hemangioendothelioam, EHE)

EHE 于 1975 年首次被提出并被认为是侵袭性的、侵犯邻近血管的支气管肺泡癌的一种形式,因此,其原名是:血管内支气管肺泡癌。不久,随着免疫组化技术的到来,使用因子Ⅷ相关性抗原进行染色,恶性细胞呈弥漫性细胞质染色,并将肺 EHE 作为内皮系肿瘤的一种。

在 1982 年,正式命名为“上皮样血管内皮瘤”,EHE 是较为罕见的血管肿瘤,为临床和组织学上介于恶性血管肉瘤和良性血管瘤之间的肿瘤。EHE 为多中心起源,肺外损伤多来自肝、骨、软组织和皮肤等器官。肺 EHE 很少见,文献报道的仅有将近 100 个案例。

肺 EHE 在无恶性肿瘤风险的患者通常表现为肺内多发结节。女性较常见,多为中年患者。在肺 EHE 诊断初患者多无症状,诊断多为偶然。在有症状患者,可常见呼吸困难、咳嗽、胸痛、咯血及体重减轻等。

肺 EHE 影像学特征性改变主要是双肺多灶性、多发性血管周结节(边界清或不清)。结节大小不等,多邻近小的或中等大小的血管及气管;也可表现为胸膜结节和渗出。肺活检很有必要,通常需开胸术、胸腔镜。

胸腔积液特征为渗出性(淋巴细胞为主)或血性,但是细胞学评估用于诊断常不足。肺 EHE 的诊断需以组织学形态为基础及免疫组化明确,其特征表现为对一些或全部的血管内皮标志物具免疫活性(CD31、CD34 和因子Ⅷ),可以从无细胞多形性、核分裂活性及具有局灶血管等鉴别 EHE 和肿瘤。

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    2022-09-17 屋顶瞄爱赏月

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    2021-11-10 chengjn
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    2021-11-10 syscxl
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