背景:医学文献中很少描述原发性恶性心脏间质瘤,只有几个公开的案例。
方法:我们介绍了一个在左心房延伸至左右下肺静脉孔的原发性恶性间叶瘤,伴有二尖瓣的机械阻塞。患者接受了手术切除和放疗。
结论:心脏肿瘤分为原发性和继发性肿瘤。原发性心脏肿瘤极为罕见,发病率低,为0.001-0.28%。原发性恶性肿瘤占它们的不到25%。恶性间膜瘤被认为是其中最罕见的。这些肿瘤增殖并可能引起转移,使它们高度恶性,预后不良,特别是如果伴有转移或侵入附近组织。
结论:心脏间叶瘤的不良预后来自于它具有侵袭性和快速生长,导致机械症状的快速进展和向附近组织的转移,这使得它被列为高度恶性肿瘤,存活率低。手术切除仍是治疗的主要手段,除了根据疾病的等级进行化疗或放疗的随访外,我们的患者就是这种情况,与以前的文献报道的情况相同。然而,需要进一步的研究和病例报告来更好地了解这种侵袭性肿瘤的最佳治疗方法.
BACKGROUND: Primary malignant cardiac mesenchymoma tumors are rarely described in the medical literature, with only a few published cases.
METHODS: We present a
case of a primary malignant mesenchymoma in the left atrium extending to the left and right inferior pulmonary vein orifices, accompanied by mechanical obstruction of the mitral valve. The patient underwent surgical removal and radiotherapy.
CONCLUSIONS: Cardiac tumors are categorized as primary and secondary tumors. Primary cardiac tumors are extremely rare, with a low incidence rate of 0.001-0.28 %. Primary malignant tumors constitute less than 25 % of them. Malignant mesenchymoma is considered the rarest of them. These tumors proliferate and may cause metastases, making them highly malignant with a poor prognosis, especially if accompanied by metastases or invasion of nearby tissues.
CONCLUSIONS: The poor prognosis of cardiac mesenchymoma comes from the fact that it is aggressive and rapidly growing, which causes a rapid progression of mechanical symptoms and metastasis to nearby tissues, which makes it classified as a highly malignant tumor with a low survival rate. Surgical resection remains the mainstay of treatment, in addition to follow-up with chemotherapy or radiotherapy according to the grade of the disease, which was the
case with our patient and the same as reported in previous literature. However, further research and
case reports are needed better to understand the optimal management of this aggressive tumor.