Kuo C S, Hsu H C, Huang C H, Liu S M, Ho C H
Department of Medicine, Veterans General Hospital-Taipei, Taiwan, R.O.C.
Zhonghua Yi Xue Za Zhi (Taipei). 1997 Feb;59(2):136-40.
Primary malignant cardiac tumors are uncommon, and cardiac leiomyosarcoma is extremely rare. We reported a case of left atrial (LA) leiomyosarcoma with unusual clinical manifestations. A 28-year-old female presented with unknown cause of fever, body weight loss and anemia for two months. Echocardiography and magnetic resonance image study disclosed a 5 x 3 x 3.6 cm3 lobulated mass in the LA with invasion to its posterior wall. Histologic and immuno-histochemical studies of the resected specimen revealed a picture of leiomyosarcoma. The patient improved after surgical resection and post-operative chemotherapy. The literature was reviewed with a discussion of the clinical manifestations, diagnosis and treatment strategy of this rare tumor. Diagnosis of LA leiomyosarcoma is frequently delayed to make a very poor prognosis. Postoperative chemotherapy should be considered because of highly possible incomplete resection. However, an optimal treatment regimen remains unknown.
原发性恶性心脏肿瘤并不常见,而心脏平滑肌肉瘤极为罕见。我们报告了一例具有不寻常临床表现的左心房平滑肌肉瘤病例。一名28岁女性出现不明原因发热、体重减轻和贫血达两个月。超声心动图和磁共振成像检查发现左心房有一个5×3×3.6立方厘米的分叶状肿块,侵犯其后壁。对切除标本进行的组织学和免疫组织化学研究显示为平滑肌肉瘤表现。患者经手术切除和术后化疗后病情好转。本文回顾了相关文献,并讨论了这种罕见肿瘤的临床表现、诊断和治疗策略。左心房平滑肌肉瘤的诊断常常延迟,导致预后很差。由于极有可能切除不完全,应考虑术后化疗。然而,最佳治疗方案仍不明确。