Larner College of Medicine, University of Vermont, Burlington, VT, USA.
Department of Pathology and Laboratory Medicine, University of Vermont Medical Center, Burlington, VT, USA.
J Cancer Res Clin Oncol. 2021 Apr;147(4):1137-1144. doi: 10.1007/s00432-021-03512-x. Epub 2021 Feb 6.
Neoplasms of the retroperitoneum that contain a major fat component may represent either benign entities, such as lipomas or angiomyolipomas, or malignancy such as liposarcoma. Distinguishing these diagnoses has important implications for management. While liposarcomas often stain positively for MDM2 and CDK4 proteins, absence of these markers can lead to diagnostic and management challenges.
We examined three cases in our institution of fat-containing masses of the retroperitoneum that lacked MDM2 and CDK4 markers to highlight the challenges in diagnosing and managing these cases. A thorough review of the literature examining radiologic and histologic features that can be used to determine that diagnosis was conducted and summarized.
The three cases we present represent the three main diagnostic entities that can be found in among fatty tumors of the retroperitoneum: lipoma, angiomyolipoma, and liposarcoma. While radiologic features and analysis of histology helped to inform management, these cases in conjunction with the literature also illustrate the limitations of the diagnostic work up and importance also factoring the biologic behavior of the tumor in its management.
Fat-containing tumors of the retroperitoneum that do not stain for MDM2 or CDK4 can pose a diagnostic challenge. Assessing radiologic and pathologic features in conjunction with the biologic behavior of these tumors should inform their management.
腹膜后含有大量脂肪成分的肿瘤可能代表良性实体,如脂肪瘤或血管平滑肌脂肪瘤,或恶性肿瘤,如脂肪肉瘤。区分这些诊断对管理具有重要意义。虽然脂肪肉瘤通常对 MDM2 和 CDK4 蛋白呈阳性染色,但这些标志物的缺失可能会导致诊断和管理方面的挑战。
我们在本院检查了三个腹膜后含有脂肪的肿块病例,这些肿块缺乏 MDM2 和 CDK4 标志物,以突出诊断和管理这些病例所面临的挑战。对用于确定诊断的影像学和组织学特征进行了全面的文献复习,并进行了总结。
我们提出的三个病例代表了腹膜后脂肪性肿瘤中可能存在的三个主要诊断实体:脂肪瘤、血管平滑肌脂肪瘤和脂肪肉瘤。虽然影像学特征和组织学分析有助于指导治疗,但这些病例以及文献还说明了诊断工作的局限性,并且在管理中还需要考虑肿瘤的生物学行为。
腹膜后含有脂肪且不染色 MDM2 或 CDK4 的肿瘤可能会带来诊断挑战。评估这些肿瘤的影像学和病理学特征以及它们的生物学行为应有助于指导其治疗。