文章摘要

1例特殊的儿童肝血管肉瘤临床病理分析

作者: 1程波, 2刘傲飞, 1赵欢, 1许琳, 1孙锁柱
1 火箭军特色医学中心病理科,北京 100080
2 火箭军特色医学中心血管神经外科,北京 100080
通讯: 孙锁柱 Email: mingzhu0368@sina.com
DOI: 10.3978/j.issn.2095-6959.2021.09.039

摘要

采用光镜、免疫组织化学等方法对1例3岁儿童肝原发组织学形态多样的血管肉瘤进行临床、病理特征分析。患者为3岁女童,肝右叶巨大包块,左叶多发大小不等结节,表面灰白灰红,触之质韧,多灶出血。组织学形态多样,包括成片血窦样区域,形态较温和的I型、II型血管内皮瘤区域,以及血管肉瘤区域。肉瘤部分也呈现出多种形态(梭形细胞实性生长、上皮样泡状核细胞片巢状分布、不规则圆形幼稚血管形成),肿瘤细胞异型显著,细胞核深染,核分裂易见。免疫组织化学染色显示:CD31、CD34、ERG、GLUT1、Vimentin弥漫阳性,Fli-1及VIII因子阴性,Ki-67指数为5%~60%。临床表现有中度贫血、甲状腺功能低下等。儿童原发性肝血管肉瘤非常少见,含有多种组织学形态(即从良性到中间型,至恶性肉瘤形态)的血管肉瘤更为罕见,其预后差。组织学检查时应广泛取材,以便将其与其他良性、中间型血管肿瘤相鉴别,做出明确诊断。
关键词: 肝脏;血管肉瘤;血管内皮瘤;儿童

Clinicopathological analysis of a special case of hepatic angiosarcoma in children

Authors: 1CHENG Bo, 2LIU Aofei, 1ZHAO Huan, 1XU Lin, 1SUN Suozhu
1 Department of Pathology, Rocket Force Medical Center, Beijing 100080, China
2 Department of Vascular Neurosurgery, Rocket Force Medical Center, Beijing 100080, China

CorrespondingAuthor: SUN Suozhu Email: mingzhu0368@sina.com

DOI: 10.3978/j.issn.2095-6959.2021.09.039

Abstract

The clinicopathological features of a 3-year-old child with multiple primary hepatic angiosarcomas were analyzed by light microscopy and immunohistochemistry, and the literature was reviewed. The patient was a 3-year-old girl with a large mass in the right lobe and multiple nodules of different sizes in the left lobe of the liver. The surface of the nodules was grayish white and grayish red, tough when touching and multifocal bleeding. The histologic pattern was varied, including areas with large sinusoids, milder forms of type I and type II hemangioendothelioma, angiosarcoma. The sarcomas also showed a variety of morphologies (solid growth of spindle cell, patch nest distribution of epithelioid follicular cell, irregular round immature blood vessel formation). The tumor cells were significantly heterogeneous, the nucleus was deeply stained and nuclear divisions were easy to see. Immunohistochemical staining showed diffuse positive for CD31, CD34, ERG, GLUT1 and Vimentin, negative for Fli-1 and factor VIII. Ki-67 index was about 5%–60%. Clinical manifestations included moderate anemia, hypothyroidism, etc. Primary hepatic angiosarcomas in children were very rare. Angiosarcomas with multiple histological morphologies (from benign to intermediate to malignant) were rarer and had a poor prognosis. It should be widely sampled and histologically distinguished from other benign and intermediate vascular tumors to make a definite diagnosis.
Keywords: liver; angiosarcoma; hemangioendothelioma; children