Agaimy Abbas, Strecker Thomas
Institute of Pathology, Friedrich-Alexander-University Erlangen-Nuremberg, Germany.
Int J Clin Exp Pathol. 2011 Mar;4(3):307-11. Epub 2011 Mar 22.
Although rare, papillary fibroelastoma (PFE) of the heart valves and atrial myxoma represent the two most common cardiac tumors. Coexistence of these two lesions has been documented in rare case reports. We describe the case of a 77-year-old man who presented with slightly progressive chest pain associated with dyspnea, fatigue and edema of the lungs. Transthoracic echocardiography detected a left atrial mass that has been successfully excised. Histopathological examination showed a neoplasm combining features of both atrial myxoma and PFE. However, close evaluation of the latter showed microscopic foci of myxomatous tissue within papillary cores, indicating that the PFE-like component has developed around preexisting myxomatous tissue that served as a nidus for papillary fronds, probably by a process of fibrinous microthrombosis, organization and endothelialisation. This unusual case may shed light on the pathogenesis of the PFE pattern.
尽管罕见,但心脏瓣膜的乳头状纤维弹性瘤(PFE)和心房黏液瘤是两种最常见的心脏肿瘤。这两种病变并存的情况在罕见的病例报告中已有记载。我们描述了一名77岁男性的病例,他出现了与呼吸困难、疲劳和肺部水肿相关的轻度进行性胸痛。经胸超声心动图检测到一个左心房肿块,该肿块已成功切除。组织病理学检查显示肿瘤兼具心房黏液瘤和PFE的特征。然而,对后者的仔细评估显示,在乳头核心内有黏液瘤样组织的微小病灶,表明PFE样成分是围绕先前存在的黏液瘤样组织发展而来的,该组织作为乳头叶的病灶,可能是通过纤维蛋白微血栓形成、机化和内皮化过程。这个不寻常的病例可能有助于揭示PFE模式的发病机制。