Booth A M, LeGallo R D, Stoler M H, Waldron P E, Cerilli L A
Department of Pathology, University of Virginia Health System, Charlottesville 22908, USA.
Pediatr Dev Pathol. 2001 Sep-Oct;4(5):490-5. doi: 10.1007/s10024001-0046-x.
We report the seventh case of angiosarcoma of the heart in a child. The patient was a 23-month-old female who presented for lower extremity limping and underwent open surgical biopsy of the femur. Immediately postoperatively, she developed pericardial tamponade, and a bulky intracardiac mass was discovered as the underlying cause. The mass was composed of highly pleomorphic tumor cells reactive for the endothelial markers CD31, CD34, and factor VIII-related antigen (FVIII-RA). Staging evaluation revealed widespread metastases involving the brain, ovaries, and bone marrow. She died of complications of metastatic disease 8 months following initial presentation. Unusual features of this case include the young age of the patient, left-sided nature of the cardiac tumor, presentation secondary to metastatic disease, and the pattern of metastases. The literature on cardiac angiosarcoma, which is limited to six case reports in the pediatric population, is also reviewed.
我们报告了第七例儿童心脏血管肉瘤病例。该患者为一名23个月大的女性，因下肢跛行就诊，并接受了股骨开放性手术活检。术后即刻，她出现了心包填塞，发现其根本原因是心脏内有一个巨大肿块。该肿块由对内皮标志物CD31、CD34和因子VIII相关抗原（FVIII-RA）呈反应性的高度多形性肿瘤细胞组成。分期评估显示广泛转移，累及脑、卵巢和骨髓。初次就诊8个月后，她死于转移性疾病的并发症。该病例的不寻常特征包括患者年龄小、心脏肿瘤位于左侧、因转移性疾病就诊以及转移模式。本文还回顾了关于心脏血管肉瘤的文献，儿科人群中仅有六例病例报告。
