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汉族人群上皮样血管内皮瘤的临床特征:一项回顾性分析。

The clinical features of epithelioid hemangioendothelioma in a Han Chinese population: A retrospective analysis.

作者信息

Guo Qinyue, Xue Jing, Xu Lin, Shi Zhihong, Zhou Bo

机构信息

Critical Care Medicine Department of Respiratory, the First Affiliated Hospital of Xi'an Jiaotong University Department of Endocrinology, the Affiliated Guangren Hospital, Xi'an Jiaotong University, Xi'an, Shaanxi, China.

出版信息

Medicine (Baltimore). 2017 Jun;96(26):e7345. doi: 10.1097/MD.0000000000007345.

Abstract

Epithelioid hemangioendothelioma (EHE) is a rare indolent vascular tumor which occurs at liver, lung, bone, and so on. However, the etiology of EHE is evasive.These patients were enrolled at the First Affiliated Hospital of Xi'an Jiaotong University from January 2011 to December 2015. Retrospective analysis is done by demographic data of clinical manifestations, laboratory parameters, CT imaging, histological, and immunohistochemical features in 9 cases in Shaanxi.Of the patients, 8 were females (88.9%) and 1 were males (11.1%). The age ranged from 34 to 71 years (mean 49 years; median 49 years). Anatomical sites of primary lesions were as follows: liver (n = 6, 66.7%), upper extremities (n = 1, 11.1%), sublingual gland (n = 1, 11.1%), and spine (n = 1, 11.1%). Metastatic disease was diagnosed in 5 cases (55.6%) with occurrence in lung (n = 4, 44.4%), bone (n = 2, 22.2%), upper extremities (n = 1, 11.1%), pleura (n = 1, 11.1%), and spleen (n = 1, 11.1%). Tumor size ranged from 0.5 to 6.8 cm (mean 3 cm). The most tumors were composed of highly cellular areas with small and prominent nucleoli in vesicular nuclei, and ERG (100%) was the most frequently positive in these cases, followed by CD31 (88.9%) and CD34 (77.8%) via histology and immunohistochemistry techniques.EHE is a very rare in Shaanxi. It is significant to find its clinical, radiological, and pathological characters, helping for EHE early diagnosis and treatment, reducing misdiagnosis and improving life quality.

摘要

上皮样血管内皮瘤(EHE)是一种罕见的惰性血管肿瘤,可发生于肝脏、肺、骨等部位。然而,EHE的病因尚不明确。这些患者于2011年1月至2015年12月在西安交通大学第一附属医院登记入组。对陕西9例患者的临床表现、实验室参数、CT影像、组织学及免疫组化特征的人口统计学数据进行回顾性分析。患者中,女性8例(88.9%),男性1例(11.1%)。年龄范围为34至71岁(平均49岁;中位数49岁)。原发灶的解剖部位如下:肝脏(n = 6,66.7%)、上肢(n = 1,11.1%)、舌下腺(n = 1,11.1%)和脊柱(n = 1,11.1%)。5例(55.6%)诊断为转移性疾病,转移部位包括肺(n = 4,44.4%)、骨(n = 2,22.2%)、上肢(n = 1,11.1%)、胸膜(n = 1,11.1%)和脾脏(n = 1,11.1%)。肿瘤大小为0.5至6.8 cm(平均3 cm)。通过组织学和免疫组化技术,大多数肿瘤由细胞丰富区组成,核呈泡状,核仁小而明显,其中ERG(100%)最常呈阳性,其次是CD31(88.9%)和CD34(77.8%)。EHE在陕西非常罕见。了解其临床、放射学和病理学特征具有重要意义,有助于EHE的早期诊断和治疗,减少误诊并提高生活质量。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0e5/5500074/3d12300b5621/medi-96-e7345-g002.jpg

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