Ho Karen, Yatham Kavya, Seno Rommel, Sultan Omar
Department of Medicine, University of Saskatchewan College of Medicine, 103 Hospital Drive, Saskatoon, SK, Canada S7N 0W8.
Department of Pathology and Laboratory Medicine, Saskatchewan Health Authority, 4101 Dewdney Avenue, Regina, SK, Canada S4T 1A5.
Eur Heart J Case Rep. 2021 Oct 7;5(11):ytab410. doi: 10.1093/ehjcr/ytab410. eCollection 2021 Nov.
Intimal sarcoma is an exceedingly rare type of primary cardiac tumour. It is characterized by poorly differentiated spindle-shaped cells that can mimic smooth muscle and is strongly associated with MDM2 genetic amplification. Owing to its rarity and non-distinctive histological features, diagnosis remains a significant challenge.
In this case report, we describe a case of primary cardiac intimal sarcoma in a 37-year-old woman who presented with atrial fibrillation (AF) and a left atrial mass. Despite having a histological sample from an excised left atrial mass, the diagnosis was not made until she presented with back pain secondary to metastatic disease to the spine.
Primary cardiac intimal sarcoma is an extremely rare diagnosis. The mainstay management of intimal cardiac sarcoma is aggressive surgical resection. Unfortunately, the prognosis of cardiac sarcomas remains very poor, with a mean survival between 3 months and 1 year. This case of cardiac intimal sarcoma highlights the difficulty in establishing a diagnosis, particularly given the unusual presentation of AF.
内膜肉瘤是一种极其罕见的原发性心脏肿瘤。其特征是分化不良的梭形细胞，可类似平滑肌，且与MDM2基因扩增密切相关。由于其罕见性和无明显的组织学特征，诊断仍然是一项重大挑战。
在本病例报告中，我们描述了一名37岁女性原发性心脏内膜肉瘤的病例，该患者表现为心房颤动（AF）和左心房肿块。尽管有切除的左心房肿块的组织学样本，但直到她因脊柱转移疾病出现背痛时才做出诊断。
原发性心脏内膜肉瘤是一种极其罕见的诊断。心脏内膜肉瘤的主要治疗方法是积极的手术切除。不幸的是，心脏肉瘤的预后仍然非常差，平均生存期在3个月至1年之间。这个心脏内膜肉瘤病例凸显了诊断的困难，特别是考虑到AF的不寻常表现。
