Durieux Rodolphe, Tchana-Sato Vincent, Lavigne Jean-Paul, Radermecker Marc A, Moonen Marie, Scagnol Irène, Gennigens Christine, Defraigne Jean-Olivier
Department of Cardiothoracic Surgery, University Hospital of Liège, Domaine Universitaire du Sart-Tilman, Liège, Belgium.
Department of Cardiology, University Hospital of Liège, Domaine Universitaire du Sart-Tilman, Liège, Belgium.
J Card Surg. 2021 Jan;36(1):357-362. doi: 10.1111/jocs.15200. Epub 2020 Nov 22.
Cardiac intimal sarcoma is extremely rare and aggressive primary malignant cardiac tumors. Here, we reported the case of a young man initially operated for a tumor of the left atrium, causing a dynamic obstruction of the mitral valve and (mis-)diagnosed as a myxoma at the histopathological analysis. Patient presented a local recurrence at 3 months and was reoperated. Pathology revealed this time the presence of an intimal sarcoma. Patient received adjuvant chemotherapy. Despite a good local control, the 1-year follow-up positron emission tomography scan revealed the presence of a metastasis in the left adrenal gland that was surgically resected. This article aims to highlight the risk of misdiagnosis in case of cardiac tumors, the hypothetical concept of malignant transformation of a cardiac myxoma, the aggressive course of the extremely rare cardiac intimal sarcoma, and the therapeutic modalities available to treat this pathology.
心脏内膜肉瘤是极其罕见且侵袭性强的原发性恶性心脏肿瘤。在此，我们报告了一名年轻男性的病例，该患者最初因左心房肿瘤接受手术，该肿瘤导致二尖瓣动态梗阻，在组织病理学分析中被（错误）诊断为黏液瘤。患者在3个月时出现局部复发并接受了再次手术。此次病理显示存在内膜肉瘤。患者接受了辅助化疗。尽管局部控制良好，但1年的随访正电子发射断层扫描显示左肾上腺存在转移灶，该转移灶已通过手术切除。本文旨在强调心脏肿瘤误诊的风险、心脏黏液瘤恶性转化的假设概念、极其罕见的心脏内膜肉瘤的侵袭性病程以及治疗该疾病的可用治疗方式。
