【呼吸系统】肺上皮样血管内皮瘤 | epithelioid hemangioendothelioma...

1

病史

36/F, Referred due to incidentally identified multiple pulmonary nodules

36岁女性，偶发多发肺结节，边界清

2

影像学检查

图1 胸片正位

图2 胸部CT平扫 肺窗及纵隔窗

图3 PET/CT

3

影像学表现

Fig 1. Chest PA shows multiple pulmonary nodules and mild elevation of right hemidiaphragm.
图1. 胸片显示两肺多发结节，右侧横膈轻微抬高。

Fig 2 Lung and mediastinal window (1.0-mm section thickness) images show multiple small well-defined nodules in both lungs. Some of the nodules show internal calcifications.
图2.  肺窗及纵隔窗（1mm层厚）显示两肺多发小结节，边界清楚。部分结节见内部钙化。

Fig 3. PET/CT images depict scanty FDG uptake in bilateral lung nodules.
图3.  PET/CT显示双侧肺结节FDG未见高摄取。

4

诊断

Pulmonary epithelioid hemangioendothelioma
肺上皮样血管内皮瘤

5

诊断要点

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare, low- to intermediate-grade tumor of endothelial origin with features of epithelioid and histiocytoid cells.
PEH是一种少见的中低度恶性内皮源性肿瘤，具有上皮样和组织细胞样细胞的特征。

There are three distinctive CT patterns:
PEH有三种特征性CT表现：

1)Multiple pulmonary nodules (mimicking hematogenous metastases), with calcifications occasionally seen within the nodules；
1) 多发肺结节（类似于血源性转移），偶发结节内钙化；

2)Multiple pulmonary reticulonodular opacities (mimicking hematolymphangitic metastases), which could be associated with interlobular septal thickening and ground-glass opacities and may show poor prognosis；

2)多发肺网格结节影（类似于血淋巴道转移），可合并小叶间隔增厚及磨玻璃影，预后差；

3)Diffuse infiltrative pleural thickening (mimicking malignant pleural mesothelioma), which is seen less frequently and predominantly in older men and shows the most aggressive clinical course；
3)弥漫性浸润性胸膜增厚(类似于恶性胸膜间皮瘤)，较少见，老年男性为主，临床病程侵袭性最强；

Surgery alone is indicated in the presence of a single pulmonary nodule or unilateral multiple nodules. Lung transplantation should be considered in patients with vascular infiltration. 
单侧肺结节或单侧多发肺结节均可手术治疗。

Various chemotherapies have been reported for metastatic PEH, with variable effectiveness.

血管浸润患者应考虑肺移植。对于转移性PEH，可行化疗，疗效不一。

参考文献：

1.Joosung Sun, Kyung Joo Park MD.  / Ajou University Medical Center

Vocabulary

epithelioid [ˌepɪ'θi:lɪɔɪd] adj.上皮的

hemidiaphragm [hemɪ'daɪəfræm]横膈

hematogenous metastases：血源性转移

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