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原发性腹膜后软组织肉瘤是一类临床上比较罕见的局部侵袭性肿瘤，其治疗效果远比四肢软组织肉瘤差。为了提高本病的临床诊断符合率，影像学的检查是必不可少的，尤其是Ｂ超、ＣＴ以及ＭＲＩ的检查，对于确定肿瘤大小、部位、与邻近器官的关系和有无肝脏及腹腔的转移，具有特殊的价值。主张通过开放活检，获取组织学诊断。手术是治疗本病的主要手段，提高本病疗效的关键是手术彻底切除肿瘤。因此，我们主张在明确本病诊断后，尽可能完全或大部分切除肿瘤，对术后复发者，也要争取再次、多次手术切除，并于术后酌情辅加放疗。
Primary retroperitoneal soft tissue sarcoma is a type of clinically rare local invasive tumor, and its therapeutic effect is far worse than soft tissue sarcoma of the limbs. In order to improve the clinical diagnostic coincidence rate of this disease, imaging examination is indispensable, especially the examination of B-ultrasonography, CT and MRI, to determine the tumor size, location, relationship with adjacent organs and whether there is liver and abdominal cavity. Transfer has special value. Advocates open biopsy for histological diagnosis. Surgery is the main method to treat this disease. The key to improving the efficacy of the disease is to completely remove the tumor. Therefore, we advocate that in the clear diagnosis of the disease, as far as possible completely or most of the removal of the tumor, for postoperative recurrence, but also to fight again, multiple surgical resection, and postoperative discretionary additional radiotherapy.