伴多发淋巴结肿大的乏脂型血管平滑肌脂肪瘤并发术后顽固性淋巴瘘1例报道及文献回顾

Minimal fat renalangiomyolipoma with multiple lymph nodes enlargement and post-operative refractory lymphatic fistula: a case report and literature review

肾血管平滑肌脂肪瘤(angiomyolipoma,AML)是泌尿系统常见的良性肿瘤,主要由脂肪组织、血管及肌肉组织构成。肾AML可同时合并结节性硬化症,但多数为散发,好发于中年女性,无明显临床症状,可由影像学检查检出。典型AML在CT平扫上具有特征性的脂肪密度,易与其他肾肿瘤相鉴别,但少部分AML含有极少量或不含有脂肪成分,平扫CT难以发现负值成分,称为乏脂型血管平滑肌瘤,其确诊依赖组织病理检查结果。另外,部分AML具有恶性表象,可累及肾周、肾窦、淋巴结、其他内脏器官及下腔静脉,进一步增加了鉴别诊断的难度。目前国内尚未见乏脂型肾血管平滑肌脂肪瘤伴发淋巴结肿大的相关报道。本文报道1例27岁女性患者,无结节性硬化症家族史,初因CT发现左肾旁团块状高密度影及颈部、腋窝、腹股沟、腹腔及腹膜后多发淋巴结肿大就诊于血液科,考虑淋巴瘤可能,为明确诊断而转入泌尿外科行腹腔镜下左肾周肿物、腹膜后淋巴结切除术和活体组织检查,术后组织病理结果提示AML。患者术后并发持续性淋巴瘘,腹膜后引流乳糜液100~200 m L/d,持续12周,予禁食、补液等保守治疗措施后成功闭瘘。本文回顾了国内外的相关病例及文献,并对伴多发淋巴结肿大的肾AML诊断、治疗和术后顽固性淋巴瘘的处理进行总结和探讨。

Renal angiomyolipoma（AML） is a common benign tumor in the urinary system,mainly composed of adipose tissue,blood vessels and muscle tissue. Renal AML is sporadic in most of patients,while a few are associated with tuberous sclerosis. Classical renal AML occurs predominantly in middleaged females. Most cases are found incidentally during imaging examinations. The fat content makes AML have unique imaging characteristics and is easy to be identified with other renal tumors. However,the amount of fat varies in each tumor. AML that contains only microscopically detectable fat and whose amount of intratumoral fat may be too small to be identified on unenhanced computed tomography（CT）images is termed minimal fat or fat-poor renal AML,which appears as a high density shadow in the renal parenchyma on unenhanced CT images. Thus,it can be difficult to distinguish it from renal cell carcinoma（RCC） on imaging. Since the imaging findings are atypical,the diagnosis depends on pathological results. In addition,a few of AML can mimic malignant neoplasms. Recent studies suggested that AML might involve to peri-renal or renal sinus fat,regional lymphatics and other visceral organs,as well as inferior vena cava,which further makes the diagnosis more difficult. However,there is currently no reports about involvement of regional limphatics in minimal fat renal AML. In this article,we report a 27-yearold female patient without family history of tuberous sclerosis,who came to visit the hematologist because a high density shadow near the left kidney was found during CT scan which was accompanied by neck,armpits,groin,abdominal cavity and retroperitoneal lymph nodes enlargement. She was suspected of lymphoma in the beginning and transferred to Department of Urology to perform laparoscopic left renal mass and retroperitoneal lymph node excision and pathological examination for a definitive diagnosis. Finally,pathologic results revealed AML. Postoperative continuous lymphatic fistula developed and the retroperitoneal drainage of chylous fluid was 100-200 m L per day,lasting for 12 weeks. The fistula was successfully closed after conservative treatment including fasting and rehydration. This article summaries and discusses the diagnosis and treatment of renal AML with lymph nodes enlargement and the management of postoperative refractory lymphatic fistula by reviewing the related cases and literature.