非结节性硬化症双肾巨大血管平滑肌脂肪瘤精准诊断及同期两侧手术治疗1例及文献复习

A case of non-tuberous sclerosis complex with giant angiomyolipoma of both kidneys treated with simultaneous resection and literature review

目的探讨双肾巨大血管平滑肌脂肪瘤的精准诊断及一切口同期手术治疗的安全性及预后。方法回顾性分析1例双肾巨大血管平滑肌脂肪瘤患者的临床病例资料。患者,女性,18岁,BMI:20.96,因“触及腹部肿物2周”入院。术前双肾增强CT诊断为双肾巨大血管平滑肌脂肪瘤,CT三维重建进一步明确肿瘤位置、血供等信息。结果排除相关禁忌后,在气管插管全麻下同期采用腹部正中纵行切口行双肾血管平滑肌脂肪瘤切除术。术中见双肾周多发血管平滑肌脂肪瘤包绕肾脏,游离后切除双肾可见外生血管平滑肌瘤,手术顺利,术后常规病理结果示:(双侧肾)血管平滑肌脂肪瘤。患者术后住院10 d,无术后出血、肾衰、漏尿等并发症发生,随访至今患者未见转移、复发征象。结论双肾巨大血管平滑肌脂肪瘤除了临床症状体征诊断,必要时建议结合基因检测、影像学重建等精准诊断,制定手术计划,且一切口同期手术治疗两侧安全、可行。

Objective To explore the accurate diagnosis and the safety and prognosis of simultaneous resection of giant angiomyolipoma of both kidneys.Methods Clinical data of a patient with giant angiomyolipoma of both kidneys were retrospectively analyzed.The 18-year-old female patient with body mass index(BMI)of 20.96 kg/m^(2) was admitted due to“palpable abdominal mass for 2 weeks”.Preoperative enhanced CT showed giant angiomyolipoma of both kidneys.Three-dimensional reconstruction of CT further clarified the tumor location,blood supply and other information.Results After exclusion of relevant contrain dications,bilateral resection was performed simultaneously with midline abdominal incision under general anesthesia.There were multiple angiomyolipomas around the kidneys.After dissociation,they were resected successfully.Routine pathological results indicated(bilateral)angiomyolipomas.During the 10-day postoperative hospitalization,no bleeding,renal failure or urinary leakage occurred.During the 12-month follow-up,no metastasis or recurrence was observed.Conclusion In addition to clinical symptoms and signs,gene detection,imaging reconstruction and other accurate diagnosis are necessary to formulate the surgical plan.Simultaneous resection of bilateral giant angiomyolipomas through one incision is safe and feasible.