新发心力衰竭掩盖巨大腹膜后脂肪肉瘤

Jose Rivas Rios, Yi-xin Zhang, M. Oye, Civan Altunkaynak, Jinous Saremian, E. Missov
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引用次数: 0

摘要

L型肉瘤是最常见的软组织肉瘤之一,占所有腹膜后肉瘤的50%。大的腹膜后肉瘤可对腹内器官和胸腔产生团块效应,产生与急性失代偿性心力衰竭(HF)类似的症状,如腹部肿胀、用力时呼吸困难和矫形呼吸。然而,新发心衰掩盖大量腹膜后脂肪肉瘤的表现是非常罕见的。在这里,我们报告了一个有趣的病例,一位老年患者在多支冠状动脉疾病的背景下,新发失代偿性心衰,静脉利尿剂治疗效果良好,但症状没有完全缓解。进一步的检查显示一个巨大的腹膜后肉瘤是病因。鉴于他的心脏功能不佳,他最初不适合做手术。然而,在接受了适当的目标导向的药物治疗后,他的射血分数(EF)恢复了,现在是手术候选人。一名78岁男性,有高血压、高脂血症和吸烟史,因进行性呼吸短促、直呼、腹胀等主诉来到急诊室。初步评估时,患者血压正常,心动过速,容量超载。实验室结果显示n端前b型利钠肽水平升高,并伴有良性综合代谢面板。胸部x线显示心脏肿大伴肺血管充血。经胸超声心动图显示EF严重降低10%-15%,左心室偏心性肥厚和全身运动不足。他对静脉利尿剂反应良好,但有持续的矫形和轻微的腹胀改善。他接受了腹部和骨盆的计算机断层扫描,发现腹膜后有一个非常大的、多分叶的肿块,尺寸为29厘米× 33厘米× 35厘米,肿块影响了腹内多个器官(图1)。计算机断层扫描引导下的肿块核心针活检证实了高分化脂肪肉瘤(WDLPS),伴有非典型梭形细胞和脂肪细胞(图2)。荧光原位杂交分析显示小鼠双分钟2 (MDM2)扩增。在额外的检查中未发现转移性疾病的证据。由于EF低,患者最初不适合手术切除,然而,经过三个月的定向药物治疗,患者的EF从10% - 15%恢复到40%。门诊缺血评估显示他患有多血管疾病,并接受了冠状动脉搭桥手术。他计划做肿瘤切除术。肉瘤是起源于间质或结缔组织的80多种不同肿瘤的异质组。2018年,软组织肉瘤约占美国所有癌症的0.8%。纯WDLS约占老年心脏病学杂志
本文章由计算机程序翻译,如有差异,请以英文原文为准。
New-onset heart failure masking a massive retroperitoneal liposarcoma
L iposarcoma is one of the most common soft tissue sarcomas, representing 50% of all retroperitoneal sarcomas. A large retroperitoneal sarcoma can exert mass effect on intraabdominal organs and the thoracic cavity producing symptoms similar to those seen in acute decompensated heart failure (HF), such as abdominal swelling, dyspnea on exertion, and orthopnea. However, the presentation with new-onset HF masking a massive retroperitoneal liposarcoma is exceedingly rare. Here, we present an interesting case of an elderly patient with new-onset decompensated HF in the setting of multi-vessel coronary artery disease who responded well to intravenous diuretic therapy without complete symptom resolution. Further workup showed a massive retroperitoneal sarcoma as the contributing etiology. Given his poor cardiac function, he was not initially a surgical candidate. However, after being placed on appropriate goal-directed medical therapy, he experienced recovery in his ejection fraction (EF) and is now a surgical candidate. A 78-year-old male with a history of hypertension, hyperlipidemia, and tobacco abuse came to the emergency room with complaints of progressive shortness of breath, orthopnea, and abdominal distention. On initial evaluation, the patient was normotensive, tachycardiac, and volume overloaded. The laboratory results showed elevated N-terminal pro-B-type natriuretic peptide level with a benign comprehensive metabolic panel. Chest X-ray revealed cardiomegaly with pulmonary vascular congestion. Transthoracic echocardiogram showed a severely reduced EF of 10%–15%, left ventricular eccentric hypertrophy and global hypokinesis. He responded well to intravenous diuretics, but had persistent orthopnea and minimal improvement of abdominal distension. He underwent computed tomography of the abdomen and pelvis which showed an extremely bulky, multi-lobulated retroperitoneal mass measuring 29 cm × 33 cm × 35 cm with mass effect on multiple intra-abdominal organs (Figure 1). Computed tomography guided core needle biopsy of the mass confirmed well-differentiated liposarcoma (WDLPS) with atypical spindle cells and adipocytes (Figure 2). Fluorescence in situ hybridization analysis revealed murine double minute 2 (MDM2) amplification. No evidence of metastatic disease was found on additional workup. The patient was not initially a candidate for surgical resection due to his low EF, however, after three months of goal-directed medical therapy, he had recovery in EF from 10%− 15% to 40%. Outpatient ischemic evaluation revealed multivessel disease and he underwent coronary artery bypass surgery. He is planned for tumor resection. Sarcomas are a heterogeneous group of over 80 different tumors arising from mesenchymal or connective tissue. In 2018, soft tissue sarcomas represented approximately 0.8% of all cancers in the United States. Pure WDLS accounts for approximately Journal of Geriatric Cardiology
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