Fernández Hernández Juan Ángel, Navarro-Barrios Álvaro, Gil Vázquez Pedro José, Gil José Emilio, Varona Garcíal Laura, López Motos David, Fernández Selles Carlos, Nieto Gemma, Frutos Bernal María Dolores, Torres Salmerón Gloria, Soria Cogollos Teresa
Cirugía General y Aparato Digestivo. Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, España.
Cirugía General y Aparato Digestivo. Hospital Clínico Universitario Virgen de la Arrixaca, Murcia, España.
Rev Esp Patol. 2021 Apr-Jun;54(2):75-84. doi: 10.1016/j.patol.2020.06.006. Epub 2020 Jul 25.
Retroperitoneal lipomas are extremely rare tumors that must be differentiated from well-differentiated liposarcomas (WD-LPS).
To summarize the evidence about giant retroperitoneal lipomas or liposarcomas; and to elaborate recommendations for their management.
A systematic literature search from January 1985 to December 2019 and a review of our own cases was performed.
Our series comprises four patients, two females and two males. The diagnosis was incidental in two cases. The medium size was 26 cm, being two cases located exclusively in the retroperitoneum, one in the inguinal region and one in the buttock via pelvic space. All cases were surgically removed being confirmed the initial diagnosis of retroperitoneal lipomas in two cases, as the rest two cases were classified as WD_LPS after MDM2/CDK4 genetic analysis. The review of the available literature plus our own cases revealed 30 cases, of which 58% were woman. Only two cases were asymptomatic. The main symptom was abdominal mass (53%) followed by abdominal pain (40,6%). The median size of the lesions was 24,9 cm with a median weight of 4.576,3 g. All cases were surgically removed, being necessary to remove contiguous organs in only four cases (12,5%).
Retroperitoneal lipoma is a rare tumor which must be differentiated from WD-LPS. This is a very difficult task, being necessary to determinate MDM2 status (by FISH or MLPA), present in liposarcoma but not in lipomas, for its correct diagnosis. The treatment must be based on a complete surgical resection with negative margins.
腹膜后脂肪瘤是极为罕见的肿瘤，必须与高分化脂肪肉瘤（WD-LPS）相鉴别。
总结有关巨大腹膜后脂肪瘤或脂肪肉瘤的证据；并阐述其治疗建议。
对1985年1月至2019年12月的文献进行系统检索，并回顾我们自己的病例。
我们的系列包括4例患者，2例女性和2例男性。2例诊断为偶然发现。肿物平均大小为26cm，2例仅位于腹膜后，1例位于腹股沟区，1例经盆腔间隙位于臀部。所有病例均行手术切除，2例确诊为腹膜后脂肪瘤，其余2例经MDM2/CDK4基因分析后分类为WD-LPS。对现有文献及我们自己的病例进行回顾，共发现30例，其中58%为女性。仅2例无症状。主要症状为腹部肿块（53%），其次为腹痛（40.6%）。病变的平均大小为24.9cm，平均重量为4576.3g。所有病例均行手术切除，仅4例（12.5%）需要切除相邻器官。
腹膜后脂肪瘤是一种罕见肿瘤，必须与WD-LPS相鉴别。这是一项非常困难的任务，为正确诊断，有必要确定MDM2状态（通过FISH或MLPA），其存在于脂肪肉瘤而非脂肪瘤中。治疗必须基于完整的手术切除且切缘阴性。
