Chen Millennie J, Makehei Shafieh, Chen Isaac, De Michele Simona, Bhanu Shiv, Wei Justin
Riverside Community Hospital, 4445 Magnolia Ave, Riverside, CA 92501, USA.
Radiol Case Rep. 2025 Jan 15;20(4):1850-1853. doi: 10.1016/j.radcr.2024.09.141. eCollection 2025 Apr.
Soft tissue sarcomas (STS) are a group of rare malignant tumors arising from mesenchymal stem cells. There are more than 60 different types of neoplasms that fall under the umbrella of STS, including tumors that originate from cartilage, adipose tissue, skeletal muscle, or connective tissue, among many other tissue types. One particular type of high-grade aggressive STS is the undifferentiated pleomorphic sarcoma (UPS), formerly known as the malignant fibrous histiocytoma (MFH). There has historically been much debate about the classification and differentiation of UPS from other types of sarcomas, which has only recently been characterized by novel methods of immunohistochemistry markers and sophisticated cytogenetics. This has left much of the literature regarding UPS largely irrelevant in context of our current classification standards. A recent retrospective analysis of 100 UPS cases revealed an incidence of only 9% for UPS originating in the retroperitoneum. We present a case of UPS originating in the retroperitoneum. In this case, a patient suffering from a large right-sided intra-abdominal mass underwent complete surgical resection. We also demonstrate some of the complexities involved in the diagnosis and treatment of a rare retroperitoneal form of UPS.
软组织肉瘤(STS)是一组源自间充质干细胞的罕见恶性肿瘤。有60多种不同类型的肿瘤属于STS范畴,包括起源于软骨、脂肪组织、骨骼肌或结缔组织等多种其他组织类型的肿瘤。一种特别具有侵袭性的高级别STS是未分化多形性肉瘤(UPS),以前称为恶性纤维组织细胞瘤(MFH)。关于UPS与其他类型肉瘤的分类和鉴别,历来存在诸多争议,直到最近才通过免疫组织化学标记物的新方法和精密的细胞遗传学得以明确。这使得许多关于UPS的文献在我们当前的分类标准下基本失去了相关性。最近一项对100例UPS病例的回顾性分析显示,起源于腹膜后的UPS发病率仅为9%。我们报告一例起源于腹膜后的UPS病例。在此病例中,一名患有右侧腹腔内巨大肿块的患者接受了完整的手术切除。我们还展示了罕见的腹膜后型UPS诊断和治疗中涉及的一些复杂性。