Objective: To summarize the experience in diagnosis and treatment of primary retroperitoneal liposarcoma. Methods: The clinic data of 73 cases of primary retroperitoneal liposarcoma were reviewed. The age of patients was 23 to 75 years old, with a median age of 53 years. Forty-five cases were treated for the first time, and 28 cases were re-treated after local recurrence. All patients underwent ultrasonography, CT or MRI before operation and were diagnosed of primary retroperitoneal liposarcoma. Results: No lymph node and distant metastasis were found in all patients. The maximum diameter of the tumor was 3-40 cm, with a median diameter of 18.9 cm. Of the 45 patients with primary treatment, 6 patients were in T2 stage and 39 in T3 stage. Of the 28 patients with retreatment after local recurrence, 4 patients were in T1, 8 in T2, and 16 in T3 stage. All the 73 patients received surgery, 7 were treated with adjuvant radiotherapy, and 3 received adjuvant chemotherapy. One hundred and ten operations were performed in 73 patients. Sixty-seven cases were diagnosed of well differentiated liposarcoma, with 60 cases of lipoma-like liposarcoma and 7 cases of sclerosing liposarcoma. Nine cases were diagnosed of dedifferentiated liposarcoma, 16 cases were myxoid liposarcoma, and 2 cases were pleomorphic liposarcoma. Sixteen cases were mixed types. A total of 69 cases were followed up for 6 months to 13 years, with an average of 4 years. Sixty-six cases obtained complete resection, 34 cases relapsed, and the recurrence rate was 51.5% (34/66). Distant metastasis occurred in 4 cases. Fourteen cases died of tumor recurrence or distant metastasis and the rest were all alive. Four cases lost follow-up after 3 months. No matter in the initial treatment group or the retreatment group, the recurrence rate of combined organ resection is lower than that of simple tumor resection, and the average recurrence time was longer. Results: Primary retroperitoneal liposarcoma was rare in clinical experience. CT is the most effective method in the diagnosis of retroperitoneal liposarcoma, however pathological examination was still the key to the diagnosis. The main treatment of retroperitoneal liposarcoma was surgical resection. Complete resection was the objective of the operation, and the combined viscera resection was performed for those with invasion of other organs. Retroperitoneal liposarcoma had the characteristics of local recurrence after operation and insensitivity to radiotherapy and chemotherapy, but the recurrent tumor can still be resected.
目的： 总结原发性腹膜后脂肪肉瘤的诊治经验。 方法： 对1999年3月至2016年8月中国医学科学院肿瘤医院收治的73例原发性腹膜后脂肪肉瘤患者进行临床资料分析。患者年龄23～75岁，平均53岁。45例为我院初次治疗，28例局部复发后再程治疗。术前均行B超、CT或者核磁共振(MRI)检查，均诊断原发性腹膜后脂肪肉瘤。 结果： 所有患者均无淋巴结及远处转移，肿瘤最大径3～40 cm，中位值18.9 cm。初治的45例患者中，T2期6例，T3期39例。再程治疗的28例患者中，T1期4例，T2期8例，T3期16例。73例患者均在我院接受手术，7例行术后放疗，3例行术后化疗。73例患者共施行110次手术，110例次手术中，诊断高分化脂肪肉瘤共67例，其中脂肪瘤样型脂肪肉瘤60例，硬化型脂肪肉瘤7例；诊断去分化脂肪肉瘤9例，黏液样脂肪肉瘤16例，多形性脂肪肉瘤2例；诊断混合型16例。69例随访6个月～13年，平均4年，其中施行完整切除手术的患者66例，全部获得随访，术后复发34例，复发率51.5%(34/66)，术后4例出现远处转移。因肿瘤复发或远处转移死亡14例，其余均健在。4例3个月后失访。无论是在初治组中，还是在再程治疗组中，联合脏器切除术的术后复发率均低于单纯肿瘤病灶切除手术，且平均复发时间更长。 结论： 原发性腹膜后脂肪肉瘤临床上较少见，CT是腹膜后脂肪肉瘤最有效的诊断方法，确诊主要依靠病理学检查，手术切除仍是目前治疗腹膜后脂肪肉瘤的主要方法，完整切除达到肉眼切除干净是手术的目标，对侵犯脏器者行联合脏器切除术。.
Keywords: Diagnosis; Primary retroperitoneal liposarcoma; Surgical treatment.