原发性腹膜后平滑肌肉瘤的诊治分析

Diagnosis and Treatment of Primary Retroperitoneal Leiomyosarcoma

摘要: 目的：探讨原发性腹膜后平滑肌肉瘤的临床特点、诊断、治疗及预后情况。方法：选取新疆维吾尔自治区第六人民医院2015年01月至2021年12月收治的原发性腹膜后平滑肌肉瘤患者19例作为研究对象，总结其临床特点、诊断、治疗方法及预后情况。结果：19例中男性3例，女性16例，年龄10~71岁(中位年龄52岁)。患者以腹痛、腹胀、腹部包块等为主要表现，CT检查可见肿瘤形态不规则，呈分叶状，病灶内部密度不均，可呈不同程度强化，且囊变和坏死较多。所有患者均行手术治疗。术后常规送病理检查，肿瘤直径3.72~11.55 cm (中位数7.7 cm)，肿瘤组织主要由梭形细胞组成，呈轻中度异型性，可见核分裂象，免疫组化示SMA、Vimentin、Desmin阳性。在19例患者中，其中侵犯周围脏器8例(42.1%)，联合脏器切除7例(36.8%)。手术时间55~550 min，平均220.74 min，中位手术时间187 min；术中出血量50~1500 mL，平均出血量325.63 mL，中位出血量200 mL。术后出现并发症2例，1例术后出现肠梗阻，经胃肠减压、灌肠等治疗措施治疗后好转。1例术后出现手术后休克，经积极抗休克等治疗后好转。余患者均顺利出院。结论：腹膜后平滑肌肉瘤临床与CT表现均无特异性，确诊需依靠病理组织学及免疫组化检查，手术完整切除是首选的治疗方式，腹膜后平滑肌肉瘤术后复发率高，预后较差；对无明显手术禁忌的复发转移病人，应积极再次手术治疗。辅助治疗的有效性尚未明确，尚需进一步探究。

Abstract: Objective: To investigate the clinical features, diagnosis, treatment and prognosis of primary retroperitoneal leiomyosarcoma. Methods: A total of 19 patients with primary retroperitoneal leiomyosarcoma admitted to the Sixth People’s Hospital of Xinjiang Uygur Autonomous Region from January 2015 to December 2021 were selected as the study subjects, and their clinical characteristics, diagnosis, treatment and prognosis were summarized. Results: Among the 19 cases, 3 were males and 16 were females, aged 10~71 years (median age 52 years). The main manifestations of the patient were abdominal pain, abdominal distension, abdominal mass, etc. CT examination showed that the tumor was irregular in shape and lobulated, with uneven density inside the lesion, which could be enhanced to varying degrees, and there were many cystic changes and necrosis. All patients underwent surgical treatment. The maximum diameter of the tumor was 3.72~11.55 cm (median 7.7cm), and the tumor tissue was mainly composed of spindle cells, presenting mild to moderate atypia. Mitotic images could be seen. Immunohistochemical staining showed positive SMA, Vimentin and Desmin. Among the 19 patients, 8 cases (42.1%) involved peripheral organs, and 7 cases (36.8%) were combined with viscerectomy. The operation time ranged from 55 min to 550 min, with an average of 220.74 min and a median of 187 min. Intraoperative blood loss was 50~1500 mL, with an average of 325.63 mL and a median of 200 mL. Postoperative complications occurred in 2 cases, and intestinal obstruction occurred in 1 case, which was improved after gastrointestinal decompression and enema. Postoperative shock occurred in 1 case, which was improved after active anti-shock treatment. The remaining patients were discharged successfully. Conclusion: The clinical and CT manifestations of retroperitoneal leiomyosarcoma are non-specific, and the diagnosis depends on histopathology and immunohistochemical examination. Complete surgical resection is the preferred treatment, and the postoperative recurrence rate of retroperitoneal leiomyosarcoma is high and the prognosis is poor. For patients with recurrence and metastasis without obvious surgical contraindications, reoperation should be performed actively. The effectiveness of adjuvant therapy is not yet clear and needs to be further explored.