Dedifferentiated liposarcoma in the extremity and trunk wall: A multi-institutional study of 132 cases by the Japanese Musculoskeletal Oncology Group (JMOG)
Dedifferentiated liposarcoma in the extremity and trunk wall: A multi-institutional study of 132 cases by the Japanese Musculoskeletal Oncology Group (JMOG)
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DOI：
10.1016/j.ejso.2022.08.024
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发表时间：
2023-02-21
期刊：
影响因子：
3.8
通讯作者：
Morioka, Hideo
中科院分区：
文献类型：
--
作者：
Morii, Takeshi;Anazawa, Ukei;Morioka, Hideo
背景：去分化脂肪肉瘤主要发生在腹膜后。由于病例少，关于四肢和躯干壁的这种实体的临床特征的信息非常有限。特别是，术前评估的意义和术中管理不同组件的原则，即，方法：采用回顾性、多中心研究方法，分析四肢或躯干壁去分化脂肪肉瘤的临床特点、治疗效果及不良预后的危险因素。在去分化型病例中，从最初表现到去分化的平均持续时间为101个月。5年局部无复发生存率、无转移生存率和疾病特异性生存率分别为71.6%、75.7%和84.7%。在32例转移患者中，15例出现肺外转移。去分化区的百分比超过87.5%，边缘/病灶内的边缘，去分化区的R1/2切除是局部复发的独立危险因素。去分化区超过36 cm（2）、法国癌症中心联合会肉瘤组III级、病灶内或边缘切除是转移的独立危险因素。去分化面积超过77 cm（2）和肺转移是疾病特异性死亡率的独立危险因素。结论：四肢和躯干壁去分化脂肪肉瘤的典型临床特征在有史以来最大的队列研究中再次得到证实。评价去分化区的分级、范围和病理切缘，以及确保足够的手术切缘，在该实体的治疗中至关重要。(c)2022爱思唯尔有限公司，BASO类似于癌症外科协会和欧洲外科肿瘤学会。All rights reserved.
Background: Dedifferentiated liposarcoma occurs predominantly in the retroperitoneum. Given the paucity of cases, information on the clinical characteristics of this entity in the extremities and trunk wall is quite limited. In particular, the significance of preoperative evaluation and principles of intraoperative management of the different components, i.e., well-differentiated and dedifferentiated areas, are still to be defined.Methods: Clinical characteristics, treatment outcomes, and risk factors for poor oncological outcomes in cases of dedifferentiated liposarcoma in the extremity or trunk wall were analyzed by a retrospective, multicentric study.Results: A total of 132 patients were included. The mean duration from the initial presentation to dedifferentiation was 101 months in dedifferentiation-type cases. The 5-year local recurrence-free survival, metastasis-free survival, and disease-specific survival rates were 71.6%, 75.7%, and 84.7%, respectively. Among 32 patients with metastasis, 15 presented with extrapulmonary metastasis. A percentage of dedifferentiated area over 87.5%, marginal/intralesional margin, and R1/2 resection in the dedifferentiated area were independent risk factors for local recurrence. Dedifferentiated areas over 36 cm(2), French Federation of Cancer Centers Sarcoma Group grade III, and intralesional or marginal resection were independent risk factors for metastasis. A dedifferentiated area over 77 cm(2) and lung metastasis were independent risk factors for disease-specific mortality.Conclusions: The typical clinical characteristics of dedifferentiated liposarcoma in the extremity and trunk wall were reconfirmed in the largest cohort ever. The evaluation of the dedifferentiated area in terms of grade, extension, and pathological margin, together with securing adequate surgical margins, was critical in the management of this entity. (c) 2022 Elsevier Ltd, BASO similar to The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.