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结节性硬化症患者双侧巨大肾血管平滑肌脂肪瘤:一例报告

Bilateral Giant Renal Angiomyolipoma in a Patient with Tuberous Sclerosis Complex: A Case Report.

作者信息

Afriansyah Andika, Yusuf Abdul M, Nusaly Harry

机构信息

Department of Surgery, Wahidin Sudirohusodo Hospital, Makassar, Indonesia.

出版信息

Acta Med Indones. 2018 Jan;50(1):61-65.

Abstract

Tuberous sclerosis complex (TSC) has several renal manifestations including angiomyolipomas (AML) and renal epithelial neoplasms. A bilateral giant renal AML is extremely rare. We report a case of giant bilateral AML and discuss the diagnosis and treatment of it. The 22-year-old man was admitted due to bilateral flank pain, gross hematuria, and abdominal fullness. He had history of epilepsy, mental retardation, and delayed development during childhood. He had angiofibroma on his face since 10 years ago. Abdominal CT and MRI revealed large lobulated heterogeneous mass with fatty content. Based on those findings, we diagnosed the patient with bilateral giant renal AML. We gave conservative management for the patient and planned to total nephrectomy on the left kidney if the continued bleeding occurred. AML associated with TSC occur more frequently as multiple lesions and grows to larger size than idiopathic AML. Bilateral giant AML, which is very rare, could be treated with conservative management if no significant hemorrhage occurred.

摘要

结节性硬化症(TSC)有多种肾脏表现,包括肾血管平滑肌脂肪瘤(AML)和肾上皮性肿瘤。双侧巨大肾AML极为罕见。我们报告一例双侧巨大AML病例并讨论其诊断和治疗。该22岁男性因双侧胁腹疼痛、肉眼血尿和腹部胀满入院。他有癫痫病史、智力发育迟缓以及儿童期发育延迟。自10年前起面部有血管纤维瘤。腹部CT和MRI显示有含脂肪成分的大的分叶状不均匀肿块。基于这些发现,我们诊断该患者为双侧巨大肾AML。我们对患者进行了保守治疗,并计划如果持续出血则对左肾进行全肾切除术。与TSC相关的AML比特发性AML更常以多发病变形式出现且长得更大。非常罕见的双侧巨大AML,如果没有明显出血,可以采用保守治疗。

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