Garraud M, Margery J, Laborde F, Saint-Blancard P, David S, Bonardel G, Héno P
Service de cardiologie et de médecine aéronautique, hôpital d'instruction des armées Percy, Clamart, France.
Rev Pneumol Clin. 2010 Oct;66(5):321-5. doi: 10.1016/j.pneumo.2010.07.014. Epub 2010 Sep 6.
In this paper, the authors report the case of a 28-year-old man with pulmonary vein leiomyosarcoma presenting subacute respiratory distress. Thoracic computed tomography and transoesophagal ultrasonographic examination of the heart suggested the diagnosis of a heart tumour revealed by the obstruction of the mitral valve and pulmonary oedema. Emergency cardiac surgery revealed the mass to be a leiomyosarcoma, probably extending from the right inferior pulmonary vein and extending into the left atrium. The clinical evolution was complicated because of a sudden local relapse. The patient underwent a second cardiac intervention involving lower right lobectomy followed by adjuvant chemotherapy with an ifosfamide-adriamycin combination. This treatment failed to control the disease and a third cardiac intervention was necessary with second-line gemcitabine-paclitaxel adjuvant chemotherapy. Further recurrences were observed with metastases first in the scalp and then in the spine and adrenal glands leading to the death of the patient 2 years after the diagnosis.
在本文中，作者报告了一例28岁男性肺静脉平滑肌肉瘤患者，该患者出现亚急性呼吸窘迫。胸部计算机断层扫描和经食管心脏超声检查提示诊断为心脏肿瘤，表现为二尖瓣梗阻和肺水肿。急诊心脏手术显示肿块为平滑肌肉瘤，可能起源于右下肺静脉并延伸至左心房。由于突然局部复发，临床病程变得复杂。患者接受了第二次心脏干预，包括右下肺叶切除术，随后采用异环磷酰胺-阿霉素联合辅助化疗。该治疗未能控制疾病，因此需要进行第三次心脏干预，并采用二线吉西他滨-紫杉醇辅助化疗。进一步观察到复发，首先转移至头皮，然后转移至脊柱和肾上腺，导致患者在诊断后2年死亡。
