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一例表现为心源性胸痛的去分化型腹膜后脂肪肉瘤罕见病例。

A Rare Case of Dedifferentiated Retroperitoneal Liposarcoma Presenting As Cardiac-Sounding Chest Pain.

作者信息

Manku Bhagat, Addingadoo Parvati, Ali Amjad

机构信息

Medical Education, University Hospital Coventry & Warwickshire, Coventry, GBR.

Medicine, University Hospital Coventry & Warwickshire, Coventry, GBR.

出版信息

Cureus. 2021 Nov 12;13(11):e19503. doi: 10.7759/cureus.19503. eCollection 2021 Nov.

Abstract

Retroperitoneal liposarcomas (RPL) are rare mesenchymal tumours with an annual incidence of 2.7 cases per million. Well-differentiated liposarcomas (WDLs) and dedifferentiated liposarcomas (DDLs) are the most common subtype. WDLs are widely known to be low-grade tumours that are less likely to metastasise and easily resected. In contrast, DDLs are high-grade aggressive metastatic tumours with mortality rates between 50% and 70%. We present an unusual case of a 47-year-old male with a background of hypertension presenting with cardiac-sounding chest pain. Initially managed as an acute coronary syndrome (ACS), he eventually underwent a CT scan which revealed a 20x18x17cm retroperitoneal complex mass with possible infiltrates to the posterior wall of the greater curvature of the stomach. Ultrasound-guided biopsy and subsequent histopathology analysis revealed DDL consistent with MDM2 amplification. This case highlights how RPL can present with diagnostic difficulties. Multidisciplinary input from haematology, surgery and specialist teams is vital to optimise patient management.

摘要

腹膜后脂肪肉瘤(RPL)是一种罕见的间叶组织肿瘤,年发病率为百万分之2.7例。高分化脂肪肉瘤(WDL)和去分化脂肪肉瘤(DDL)是最常见的亚型。众所周知,WDL是低级别肿瘤,转移可能性较小且易于切除。相比之下,DDL是高级别侵袭性转移性肿瘤,死亡率在50%至70%之间。我们报告一例不寻常病例,一名47岁男性,有高血压病史,出现类似心脏病发作的胸痛。最初按急性冠状动脉综合征(ACS)进行处理,最终他接受了CT扫描,结果显示一个20×18×17cm的腹膜后复杂肿块,可能已浸润至胃大弯后壁。超声引导下活检及随后的组织病理学分析显示为与MDM2扩增一致的DDL。该病例突出了RPL在诊断上可能存在的困难。血液学、外科和专科团队的多学科协作对于优化患者管理至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/001d/8589002/f9d88b531fa8/cureus-0013-00000019503-i01.jpg

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