Department of Orthopaedic Surgery, Seoul National University Hospital, Seoul, Korea.
Department of Translational Medicine, Seoul National University College of Medicine, Seoul, Korea.
BMC Cancer. 2024 Oct 10;24(1):1259. doi: 10.1186/s12885-024-13021-y.
Dedifferentiated liposarcoma of the extremities (DDL-E) is rare in comparison to that of the retroperitoneum. Its clinical features and surgical principle for resection margins at the dedifferentiated and the well-differentiated components are yet to be elucidated.
This retrospective multi-center study examined patients diagnosed with DDL-E from August 2004 to May 2023 at 5 sarcoma centers. Clinical features, oncologic outcomes, and prognostic factors were analyzed.
A total of 107 patients were reviewed. The 5-year local recurrence free survival (LRFS), metastasis-free survival (MFS) and disease specific survival (DSS) were 84.7%, 78.6%, and 87.8%, respectively. Other primary malignancies and extrapulmonary metastasis were observed in 27 and 4 patients, respectively. The independent risk factor for local recurrence was R1/2 margin at the dedifferentiated component of the tumor. Metastasis was associated with tumor size in univariate analysis. The independent risk factor for DSS was tumor grade. Previous unplanned excision, de novo presentation, tumor depth, absence of the well-differentiated component, infiltrative border, R1/2 margin at the well-differentiated component were not associated with oncologic outcomes.
This is the largest study examining DDL-E to-date. Localized DDL-E has low potential for metastasis and carries an excellent prognosis. Other primary malignancy and extrapulmonary metastasis are more frequent in DDL-E, thus close monitoring of other sites during follow-up is recommended. While wide resection margin is the standard surgical approach for DDL-E, further investigation into moderated wide resection margin at the well-differentiated component is warranted.
与腹膜后相比，肢体去分化脂肪肉瘤（DDL-E）较为罕见。其临床特征和去分化及分化良好成分的切缘切除范围的手术原则仍有待阐明。
本回顾性多中心研究分析了 2004 年 8 月至 2023 年 5 月期间 5 个肉瘤中心诊断为 DDL-E 的患者。分析了临床特征、肿瘤学结果和预后因素。
共回顾了 107 例患者。5 年局部无复发生存率（LRFS）、无转移生存率（MFS）和疾病特异性生存率（DSS）分别为 84.7%、78.6%和 87.8%。其他原发性恶性肿瘤和肺外转移分别在 27 例和 4 例患者中观察到。肿瘤去分化成分切缘为 R1/2 是局部复发的独立危险因素。在单因素分析中，转移与肿瘤大小有关。肿瘤分级是 DSS 的独立危险因素。既往无计划切除、初发、肿瘤深度、无分化良好成分、浸润性边界、分化良好成分切缘为 R1/2 与肿瘤学结果无关。
这是迄今为止研究 DDL-E 的最大规模研究。局限性 DDL-E 转移潜力低，预后良好。DDL-E 中其他原发性恶性肿瘤和肺外转移更为常见，因此建议在随访期间密切监测其他部位。虽然广泛切除边缘是 DDL-E 的标准手术方法，但需要进一步研究在分化良好成分适度广泛切除边缘。
