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群体中:多发左房未分化多形性肉瘤

Among the Masses: Multiple Left Atrial Undifferentiated Pleomorphic Sarcomas.

作者信息

Yager Kevin, Fan Jerry, Sanford Corry B, Pourfarrokh Niloufar, Nguyen Vinh

机构信息

Department of Internal Medicine, Baylor Scott & White Medical Center, Temple, TX.

Division of Cardiology, Baylor Scott & White Medical Center, Temple, TX.

出版信息

Ochsner J. 2024 Winter;24(4):279-283. doi: 10.31486/toj.23.0143.

DOI:10.31486/toj.23.0143
PMID:39720829
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11666110/
Abstract

Undifferentiated pleomorphic sarcoma, an exceedingly rare and aggressive primary cardiac tumor arising from mesenchymal stem cells, is associated with poor prognosis and high mortality despite adequate treatment. A 52-year-old female presented with a 2-month history of angina and dyspnea on exertion. Her clinical history included severe acute respiratory syndrome coronavirus 2 myocarditis and iron deficiency anemia. Elevated troponin 1 and D-dimer prompted further investigation, and diagnostic imaging revealed multiple hypodensities in the left atrium and a right-sided pleural effusion that were causing severe mitral stenosis and pulmonary hypertension. Full body positron emission tomography scan suggested metastatic disease, prompting surgical resection of the atrial masses. Pathology confirmed high-grade undifferentiated pleomorphic sarcoma. Treatment with chemotherapy resulted in clinical stability without radiographic evidence of recurrence at 9 months. At follow-up >2 years after the initial diagnosis, echocardiogram demonstrated normal left ventricular systolic function with ejection fraction of 55% to 60%, no mitral gradient, and resolution of pulmonary hypertension. Left atrial masses are a diagnostic challenge because of the location and the technical difficulty of biopsy. Undifferentiated pleomorphic sarcoma has a 5-year survival rate of approximately 60%, so the condition necessitates prompt diagnosis and treatment.

摘要

未分化多形性肉瘤是一种极其罕见且侵袭性强的原发性心脏肿瘤,起源于间充质干细胞,尽管治疗充分,但其预后较差且死亡率高。一名52岁女性患者有2个月的劳力性心绞痛和呼吸困难病史。她的临床病史包括严重急性呼吸综合征冠状病毒2型心肌炎和缺铁性贫血。肌钙蛋白1和D - 二聚体升高促使进一步检查,诊断性影像学检查显示左心房有多个低密度影以及右侧胸腔积液,这些导致了严重的二尖瓣狭窄和肺动脉高压。全身正电子发射断层扫描提示转移性疾病,促使对心房肿块进行手术切除。病理证实为高级别未分化多形性肉瘤。化疗治疗使病情临床稳定,9个月时影像学检查无复发迹象。在初次诊断后2年多的随访中,超声心动图显示左心室收缩功能正常,射血分数为55%至60%,无二尖瓣压差,肺动脉高压消退。由于左心房肿块的位置和活检的技术难度,其诊断具有挑战性。未分化多形性肉瘤的5年生存率约为60%,因此该病需要及时诊断和治疗。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/ec85bda9246a/toj-23-0143-figure4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/443313607c08/toj-23-0143-figure1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/1ed8ae014a0c/toj-23-0143-figure2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/4a8b3fa0a544/toj-23-0143-figure3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/ec85bda9246a/toj-23-0143-figure4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/443313607c08/toj-23-0143-figure1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/1ed8ae014a0c/toj-23-0143-figure2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/4a8b3fa0a544/toj-23-0143-figure3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/236c/11666110/ec85bda9246a/toj-23-0143-figure4.jpg

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