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心脏炎性肌纤维母细胞瘤导致的突发性意外死亡:病例报告及文献复习。

Sudden unexpected death due to inflammatory myofibroblastic tumor of the heart: a case report and review of the literature.

机构信息

Department of Pathology, University of Maryland School of Medicine, Baltimore, USA.

出版信息

Int J Legal Med. 2011 Jan;125(1):81-5. doi: 10.1007/s00414-010-0496-7. Epub 2010 Jul 31.

Abstract

Inflammatory myofibroblastic tumor (IMT) or inflammatory pseudotumor is a rare primary cardiac tumor that may result in sudden death. We report a sudden unexpected death due to occlusion of the coronary arteries by IMT arising from the left coronary cusp of the aortic valve. An 8-year-old child suddenly woke up from his sleep with complaint of severe chest pain to his parents, and shortly he became unresponsive. He expired 40 min later in the hospital despite resuscitation efforts. The postmortem examination revealed a 2.5 × 2 × 1-cm mass composed of multiple entangled slender cylindrical fronts, filling the coronary sinus and obstructing the coronary ostia. The patient had complained of recurrent chest pains about 2 weeks prior to his death. Echocardiogram was conducted on the patient but did not recognize the mass. Histological examination of the mass established the diagnosis of primary cardiac IMT. The detailed pathological findings are described. In addition, the literature is reviewed, and pathogenesis, clinical presentation, and the importance of forensic autopsy examination are discussed.

摘要

炎性肌纤维母细胞瘤(IMT)或炎性假瘤是一种罕见的原发性心脏肿瘤,可能导致猝死。我们报告了一例因左冠状动脉瓣起源于主动脉瓣的炎性假瘤导致冠状动脉阻塞而突然意外死亡的病例。一名 8 岁儿童在睡眠中突然醒来,向父母诉说剧烈胸痛,不久后失去意识。尽管进行了复苏努力,他仍在 40 分钟后在医院去世。尸检显示,一个 2.5×2×1 厘米大小的肿块由多个缠绕的细长圆柱形突起组成,充满冠状窦并阻塞冠状动脉口。患者在死亡前约 2 周反复出现胸痛。对患者进行了超声心动图检查,但未识别出肿块。肿块的组织学检查确立了原发性心脏 IMT 的诊断。详细的病理发现如下。此外,还对文献进行了复习,并讨论了发病机制、临床表现和法医尸检检查的重要性。

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