Lai W Y, Young M S, Ding Y A
Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1994 Nov;54(5):329-35.
Primary malignant cardiac tumor is a rare disorder which has a myriad of clinical manifestations. It dominates about one-fourth of all cardiac tumors, exhibiting typically malignant histological characteristics and invasive behavior.
Seven cases of primary malignant cardiac tumor were collected over eight years (1985-1993) in this hospital. Each case had different manifestations. All cases were diagnosed by physical examination, echocardiogram and were subsequently proved by tissue pathology.
The majority of these seven cases of primary malignant cardiac tumor had clinical symptoms of chest distress, shortness of breath and paroxysmal nocturnal dyspnea. The relatively young ages of the patients ranged from 11 to 62 years. One case of angiosarcoma, one case of fibrosarcoma, one case of leiomyosarcoma and three cases of lymphoma had the characteristics of cardiac hemodynamic obstruction which was induced by location and size of the tumors in the heart. Five cases of those primary malignant cardiac tumor were mortal; the average life span was nine months following surgical resection and chemotherapy.
From a clinical viewpoint, primary malignant cardiac tumor characteristically displays a rapid downhill course. Death most often occurs from a few weeks to two years after the onset of symptoms. Because these tumors are rare and have a poor clinical outcome, early diagnosis and treatment are vital.
原发性恶性心脏肿瘤是一种罕见疾病，有多种临床表现。它约占所有心脏肿瘤的四分之一，通常表现出恶性组织学特征和侵袭性行为。
本医院在八年（1985 - 1993年）间收集了7例原发性恶性心脏肿瘤病例。每例表现各异。所有病例均通过体格检查、超声心动图诊断，随后经组织病理学证实。
这7例原发性恶性心脏肿瘤多数有胸闷、气短和阵发性夜间呼吸困难等临床症状。患者年龄相对较轻，介于11至62岁之间。1例血管肉瘤、1例纤维肉瘤、1例平滑肌肉瘤和3例淋巴瘤具有因肿瘤在心脏中的位置和大小导致的心脏血流动力学梗阻特征。这些原发性恶性心脏肿瘤中有5例死亡；手术切除及化疗后平均生存期为9个月。
从临床角度看，原发性恶性心脏肿瘤通常呈现病情迅速恶化的过程。死亡大多发生在症状出现后的几周至两年内。由于这些肿瘤罕见且临床预后不佳，早期诊断和治疗至关重要。
