Blachman-Braun Ruben, Aboitiz-Rivera Carlos Manuel, Aranda-Fraustro Alberto, Ransom-Rodríguez Adrián, Baltazares-Lipp Mario Enrique, Catrip-Torres Jorge Manuel, Martínez-Reding Jesús Octavio
Centro de Investigación en Ciencias de la Salud, Facultad de Ciencias de la Salud, Universidad Anáhuac México Norte, Tlalpan, Mexico City.
Echocardiography and Hemodynamics Service, Instituto Nacional de Enfermedades Respiratorias, Tlalpan, Mexico City.
Rare Tumors. 2017 Mar 28;9(1):6669. doi: 10.4081/rt.2017.6669. eCollection 2017 Mar 24.
Primary cardiac malignancies are rare entities. Although sarcomas enclosed the main group of malignant heart neoplasms, primary cardiac leiomyosarcomas are extremely rare and constitutes less than 8% of cardiac tumors. Leiomyosarcoma usually originates from the pulmonary veins and have a worm-like shaped structure. In this article, we present a case of a 40-year-old Hispanic man diagnosed with a cardiac tumor who underwent surgical resection, during pathological examination the tissue samples were consistent with a malignancy of mesenchymal origin that contained irregular bundles of spindle cells. Subsequent immunohistochemical study categorized the mass as a primary heart leiomyosarcoma with positive smooth muscle actin and muscle specific actin. As usual in this type of malignancy, patient's clinical status declined overtime, recurrence was diagnosed two months after surgery, and four months after the procedure the patient was discharge for palliative care.
原发性心脏恶性肿瘤是罕见的疾病。虽然肉瘤是恶性心脏肿瘤的主要类型，但原发性心脏平滑肌肉瘤极为罕见，占心脏肿瘤的比例不到8%。平滑肌肉瘤通常起源于肺静脉，呈蠕虫状结构。在本文中，我们报告了一例40岁的西班牙裔男性，被诊断患有心脏肿瘤并接受了手术切除，在病理检查中，组织样本与间叶源性恶性肿瘤一致，其中含有不规则的梭形细胞束。随后的免疫组织化学研究将该肿块归类为原发性心脏平滑肌肉瘤，平滑肌肌动蛋白和肌肉特异性肌动蛋白呈阳性。与这类恶性肿瘤的常见情况一样，患者的临床状况随时间推移而恶化，术后两个月诊断出复发，术后四个月患者因姑息治疗出院。
