Chua Huey Bing, Hussain Rizuana Iqbal, Shukor Nordashima Abd, Fam Xeng Inn
Urology Unit, Department of Surgery, Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan Malaysia, Kuala Lumpur, Malaysia.
Department of Radiology, Faculty of Medicine, Universiti Kebangsaan Malaysia Medical Centre, Kuala Lumpur, Malaysia.
Front Oncol. 2025 Mar 17;15:1489833. doi: 10.3389/fonc.2025.1489833. eCollection 2025.
Retroperitoneal liposarcoma, especially dedifferentiated liposarcoma (DDL), is a rare tumor type primarily affecting middle-aged and older adults in the retroperitoneum or proximal extremities. This case report highlights an exceptionally large retroperitoneal DDL that had enveloped the entire right kidney and had adhered to nearby tissues. Diagnosing retroperitoneal liposarcoma is challenging due to its asymptomatic nature until it reaches a substantial size. Imaging, particularly contrast-enhanced computed tomography (CECT), play a vital role in diagnosis, staging, and preoperative planning. Surgical resection, with the goal of R0 resection, remains the cornerstone of treatment, albeit this can be challenging due to tumor location. First-line treatment for advanced DDL involves anthracycline-based therapy. Eribulin and pazopanib show promise in second-line treatment. Ongoing clinical trials suggest a shift towards multimodal therapy. This case report reports the largest retroperitoneal liposarcoma and underscores the complexity of managing retroperitoneal DDL.
腹膜后脂肪肉瘤，尤其是去分化脂肪肉瘤（DDL），是一种罕见的肿瘤类型，主要影响中老年患者，好发于腹膜后或四肢近端。本病例报告重点介绍了一例异常巨大的腹膜后去分化脂肪肉瘤，该肿瘤包裹了整个右肾并与附近组织粘连。由于腹膜后脂肪肉瘤在体积增大之前通常无症状，因此其诊断具有挑战性。影像学检查，尤其是增强计算机断层扫描（CECT），在诊断、分期及术前规划中起着至关重要的作用。以R0切除为目标的手术切除仍然是治疗的基石，尽管由于肿瘤位置的原因，这可能具有挑战性。晚期去分化脂肪肉瘤的一线治疗包括蒽环类药物治疗。艾瑞布林和帕唑帕尼在二线治疗中显示出前景。正在进行的临床试验表明治疗正朝着多模式治疗转变。本病例报告报道了最大的腹膜后脂肪肉瘤，并强调了腹膜后去分化脂肪肉瘤治疗的复杂性。
