前纵隔上皮样血管内皮瘤CT表现与病理相关征象分析

Analysis of Pathological and CT Features of Anterior Mediastinum Epithelioid Hemangioendothelioma

目的 分析前纵隔上皮样血管内皮瘤临床、病理及CT特征,提高对本病术前诊断水平。方法 通过分析1990-2019年PUBMED及中国知网收录的具有完整病理及CT资料的前纵隔上皮样血管肉瘤,收集患者临床资料(性别、年龄)、病理(免疫组织化学、化生骨、破骨细胞样巨细胞、脂肪细胞)、CT征象(肿块密度及与周围血管的关系),并比较分析CT征象与病理的相关性。结果 共收集17例患者,其中男性8例,女性9例,发病年龄18~79岁(48±15.49),肿瘤最大长径3.3cm~20cm(6.74 cm±4.12cm)。免疫组织化显示CD31阳性64.7%、CD34阳性70.6%、Vimentin阳性47%、Ⅷ阳性41.2%;CT显示肿块高密度12例(70%)、脂肪密度7例(41%),病理组织学显示含有化生骨8例CT均表现为高密度,而另外4例CT表现为高密度患者(1例病理提示软骨粘液样基质,3例提示有钙化组织)。病理提示含有破骨细胞样巨细胞5例,其中只有3例CT表现为高密度,然而这3例患者均合并化生骨,CT高密度与化生骨有明显相关性(P<0.05)而与破骨细胞样巨细胞无明显相关性(P>0.05)。CT显示7例脂肪密度患者其中3例细胞学显示有成熟脂肪细胞,而另外4例细胞学未提及脂肪细胞。13例提及周围血管关系,来源于头壁静脉6例(46%)、上腔静脉3例(23%),主动脉弓旁3例(23%)、胸内静脉1例(8%)。11例提及术前CT诊断均误诊(100%),误诊为畸胎瘤5例CT均表现为含有高密度和脂肪密度的肿块,其余6例误诊为胸腺瘤。结论 前纵隔EHE容易误诊为畸胎瘤及胸腺瘤;肿瘤多起源于大血管,因化生骨和钙化的原因导致CT肿块内多发高密度影,并且肿块内含有成熟脂肪细胞;术前CT诊断结合肿块发病部位、密度及与周围血管关系,可能提高其术前诊断水平。

Objective To improve the rates of diagnosis for epithelioid hemangioendothelioma on anterior mediastinum, we analyzed the literature concerning the clinical, pathological and CT signs of this disease. Methods By analyzing the integrative pathology of this disease from PUBMED and CNKI literature from 1990 to 2019, we compiled the clinical data of patients including gender, age, locations of the disease, clinical symptoms and pathology results including immunohistochemistry, metaplastic bone, osteoclastlike giant cells and adipocytes, CT signs of mass density and the relationship with peripheral blood vessels. We also analyzed pathological relationships of epithelioid hemangioendothelioma with other conditions. Results This study analyzed the clinical results of 17 patients diagnosed with epithelioid hemangioendothelioma. The diameter of the cancers varied from 3.3cm to 20cm(6.7 ± 4.1cm). The cancers were all located on the upper anterior mediastinum. Immunohistochemistry showed a 64.7% level of CD31 positive cells and a 70.6% level of CD34 positive cells. Vimentin was positive in 47% of cases, and VIII was positive in 41.2% of cases. The results of CT showed hyperdense in twelve patients(70%) and fat density in seven patients(41%). The pathological histology showed metaplastic bone in eight patients, and the results of CT showed that there was calcification in four patients. The pathological findings demonstrated that six patients had osteoclast-like giant cells;four patients showed calcification on CT and two cases had no calcification. However, four cases of calcification had metaplastic bone. There was a significant correlation between CT hyperdense and the metaplastic bone(P<0.05). The results of ten patients indicated that preoperative diagnosis was all misdiagnosed(100%). Five patients diagnosed with teratoma were misdiagnosed with hyperdense and adipocytes on CT, six patients were misdiagnosed with thymoma without hyperdense and adipocytes, and one tumor was misdiagnosed as a malignant thymoma. Conclusions Anterior mediastinal EHE is easily misdiagnosed as teratoma and thymoma. Most tumors originate from large blood vessels. Due to the metaplastic bone, calcification and mature adipocytes, so hyperdense and fat-dens shown in CT were common in the tumor. In order to improve preoperative diagnostic levels, clinicians should pay attention to the preoperative CT showing the location, the density of the tumors and the relationship with the adjacent blood vessels.