Tsutsumi Koji, Aida Yoshio, Ohno Tsutomu, Ookura Mikihiko
Division of Cardiovascular Surgery, Cardiovascular Center, Sagamihara Kyodo Hospital, Sagamihara, Kanagawa, Japan.
Jpn J Thorac Cardiovasc Surg. 2005 Aug;53(8):458-62. doi: 10.1007/s11748-005-0086-7.
Primary cardiac rhabdomyosarcoma is rare. A dismal prognosis is usually given due to its high propensity for distant metastases, with survival rarely exceeding 2 years. We report a case that was initially considered to be a cardiac metastatic tumor from a primary uterine leiomyosarcoma, but after morphological and immunohistochemical examinations was shown to be a primary cardiac rhabdomyosarcoma. The right atrium was filled with a mass that oscillated between the right atrium and ventricle through the tricuspid valve. To reduce the risk of sudden death from tumor embolism into the pulmonary arteries, the tumor was resected. Even though the surgery was not curative, it eliminated the risk of sudden death due to tumor embolism into the pulmonary arteries and improved short-term survival.
原发性心脏横纹肌肉瘤很罕见。由于其远处转移倾向高,通常预后不佳,生存期很少超过2年。我们报告一例最初被认为是原发性子宫平滑肌肉瘤的心脏转移瘤,但经形态学和免疫组化检查后显示为原发性心脏横纹肌肉瘤。右心房充满了一个肿块,该肿块通过三尖瓣在右心房和心室之间摆动。为降低肿瘤栓子进入肺动脉导致猝死的风险,对肿瘤进行了切除。尽管手术不能治愈,但它消除了肿瘤栓子进入肺动脉导致猝死的风险,并改善了短期生存。