腎臟惡性類上皮血管肌肉脂肪瘤:病例報告
侯俊名 劉忠一 林柏宏 張英勛 莊正鏗 馮思中
林口長庚紀念醫院外科部泌尿外科
Malignant renal epithelioid angiomyolipoma:case report
Chun- Ming Hou, Chung-Yi Liu, Poh-Hung Lin , Yin-Hsu Chang, Cheng-Keng Chuang Szu-Chung Feng
Division of Urology, Department of Surgery, Linkou Chang Gung Memorial Hospital, Department of Urology, Taoyuan, Taiwan
Abstract:
We reported a 56 years old male patient developed significant body weight loss in 2 months. Right renal mass was noted during abdominal ultrasound examination. Then he was referred to our urology department for further management.Abdominal CT was arranged and showed huge right renal mass about 13 cm with inferior vena cava tumor thrombus. We arranged combined surgery with cardiovascular surgeon. The right radical nephrectomy and inferior vena cava exploration with tumor excision were performed. The pathology report revealed right renal malignant epithelioid angiomyolipoma with negative margin.
The patient discharged without any complication 10 days after operation. Malignant renal angiomyolipoma is rare in Taiwan even worldwide. The radical nephrectomy alone may be inadequate due to the highly aggressive nature of epithelioid angiomyolipoma. So adjuvant therapy with closely follow up after operation should consider. Chemotherapy with doxorubicin and cisplatin had been reported. The target therapy including mammalian target of rapamycin (mTOR) inhibitor and sorafenib were also reported.
Further study should be continued to conduct a standard protocol for the adjuvant therapy of malignant epithelioid angiomyolipoma.