Sasaki K, Tanaka S, Ikeshita M, Shoji T, Fujita Y, Katagiri K
Department of Thoracic Surgery, Nihon Medical School, Tokyo, Japan.
Nihon Kyobu Geka Gakkai Zasshi. 1991 Dec;39(12):2240-5.
Malignant fibrous histiocytoma (MFH) has been rarely reported as a primary tumor of the heart. We present a case of a primary intracardiac MFH and review the 28 previous reports of this rare cardiac tumor. A 48-year-old woman underwent an operation for congestive heart failure (CHF) due to a left atrial tumor. A large multinodular tumor attached to the posterior wall of the left atrium and extended into the mitral annulus and the posterior leaflet of the mitral valve. The tumor was resected incompletely and mitral valve replacement was performed. Pathological diagnosis was MFH. The patient did well in the postoperative period, but she again presented with symptoms of CHF four months later. Chest CT and two-dimensional echocardiogram demonstrated a recurrent tumor of the left atrium. Since the tumor grew rapidly despite chemotherapy, a reoperation was performed to remove it. Nevertheless, as the tumor was found to have extended into the pericardial cavity and posterior mediastinum, only partial resection was possible. After a protracted operation, the heart failed to sustain the circulation and the patient died. The prognosis of intracardiac MFH was poor in spite of surgery, radiation and/or chemotherapy. However, repeated surgical treatments can relieve symptoms of CHF and may prolong the life of patients.
恶性纤维组织细胞瘤（MFH）作为原发性心脏肿瘤鲜有报道。我们报告一例原发性心脏内MFH病例，并回顾此前关于这种罕见心脏肿瘤的28篇报道。一名48岁女性因左心房肿瘤导致充血性心力衰竭（CHF）而接受手术。一个大的多结节肿瘤附着于左心房后壁，并延伸至二尖瓣环和二尖瓣后叶。肿瘤未完全切除，并行二尖瓣置换术。病理诊断为MFH。患者术后恢复良好，但4个月后再次出现CHF症状。胸部CT和二维超声心动图显示左心房复发肿瘤。尽管进行了化疗，肿瘤仍迅速生长，因此再次手术切除。然而，由于发现肿瘤已延伸至心包腔和后纵隔，只能进行部分切除。经过长时间手术，心脏无法维持循环，患者死亡。尽管进行了手术、放疗和/或化疗，心脏内MFH的预后仍然很差。然而，重复的手术治疗可以缓解CHF症状，并可能延长患者生命。
