Gabbieri Davide, Rossi Giulio, Bavutti Lucilla, Corghi Fabrizio, Zacà Fabio, Sarandria Domenico, Pierangeli Angelo, Ghidoni Italo
Divisions of Cardiovascular Surgery, Hesperia Hospital, Modena, Italy.
J Cardiovasc Med (Hagerstown). 2006 May;7(5):373-8. doi: 10.2459/01.JCM.0000223263.77674.db.
Papillary fibroelastoma is the most common primary tumour of cardiac valves, with the potential for embolic events and obstructive effects. Location in non-valvular endocardium is extremely rare. Transthoracic and transoesophageal echocardiography have greatly increased the ability to make the diagnosis of these surgically treatable tumours in a timely fashion. We report the case of a 70-year-old woman with symptoms and ventilation-perfusion scan evidence of pulmonary embolization from a papillary fibroelastoma of the right atrium. Initial transthoracic echocardiography failed to identify the tumour, whereas transoesophageal echocardiography demonstrated a mobile echodense mass attached to the right atrial free wall. After surgical excision, histopathology revealed papillary fibroelastoma. The epidemiology, aetiology, localization, macroscopy, histopathology, immunohistochemistry, clinical presentation, diagnosis and management of cardiac papillary fibroelastoma are reviewed. The case is unusual with respect to the site of origin of the papillary fibroelastoma as well as its clinical presentation, which is clearly related to pulmonary embolization.
乳头状纤维弹性瘤是心脏瓣膜最常见的原发性肿瘤，具有发生栓塞事件和阻塞效应的可能性。位于非瓣膜性心内膜极为罕见。经胸和经食管超声心动图极大地提高了及时诊断这些可手术治疗肿瘤的能力。我们报告一例70岁女性，有症状且通气灌注扫描显示存在源于右心房乳头状纤维弹性瘤的肺栓塞。最初经胸超声心动图未能发现肿瘤，而经食管超声心动图显示一个附着于右心房游离壁的活动回声密集团块。手术切除后，组织病理学显示为乳头状纤维弹性瘤。本文对心脏乳头状纤维弹性瘤的流行病学、病因、定位、大体检查、组织病理学、免疫组织化学、临床表现、诊断和治疗进行了综述。该病例在乳头状纤维弹性瘤的起源部位及其临床表现方面都不常见，其临床表现显然与肺栓塞有关。
