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目的探讨腹膜后去分化脂肪肉瘤(dedifferentiated liposarcoma,DDL)的临床病理学特点。方法回顾性分析16例腹膜后去分化脂肪肉瘤的临床表现、影像学资料、光学显微镜形态和免疫学表型,并对患者进行随访。结果 16例中为复发再次手术者10例。所有患者肿瘤均发生于腹膜后。CT能够清楚地显示肿瘤的生长范围及对周围脏器的推挤、浸润,影像学具有一定的提示作用。镜下观察显示去分化脂肪肉瘤含有分化性成分和去分化成分,去分化成分可表现为纤维肉瘤样、恶性纤维组织细胞瘤样、恶性外周神经鞘瘤样、平滑肌肉瘤样、横纹肌肉瘤样、血管肉瘤样、血管外皮瘤样等。肿瘤可以以一种成分为主,也可以几种成分同时存在,免疫表型表达各异。16例患者均通过手术治疗,其中9例为单纯肿物切除,7例同时切除了肿物及周围粘连的脏器。随访至2012年1月(随访3-24月)均无复发及转移。结论去分化脂肪肉瘤中的去分化成分表现各异,低级别和高级别区域可在同一肿瘤内并存。对于复发病例仍然应该争取再次手术切除,以缓解症状,延长生存期。
Objective To investigate the clinicopathological features of dedifferentiated liposarcoma (DDL). Methods Retrospective analysis of 16 cases of retroperitoneal dedifferentiated liposarcoma clinical manifestations, imaging data, optical microscopy and immunological phenotype, and patients were followed up. Results of 16 cases of recurrence re-operation in 10 cases. All patients had tumors in the retroperitoneum. CT can clearly show the tumor growth and the surrounding organs of the push, infiltration, imaging has some tips. Microscopic examination showed that the dedifferentiated liposarcoma contains differentiated components and dedifferentiated components, dedifferentiated components can be expressed as fibrosarcoma-like, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumor-like, leiomyosarcoma-like, rhabdomyosarcoma-like, vascular Sarcomatoid like, vascular endothelial tumor-like and so on. Tumor can be a component-based, but also several components exist at the same time, the immune phenotype expression varies. All 16 patients were surgically treated, including 9 cases of simple tumor resection and 7 cases of resection of the tumor and surrounding organs. Follow-up to January 2012 (followed up for 3-24 months) showed no recurrence and metastasis. Conclusions The dedifferentiated components in dedifferentiated liposarcoma show different performance. The low-grade and high-grade regions can co-exist in the same tumor. Recurrence cases should still fight for another surgical resection to relieve symptoms and prolong survival.