黃品叡 1、沈正煌 1、周詠欽 1,2、林昌德 1、鄭明進 1
1 嘉義基督教醫院 外科部 泌尿科 2 亞洲大學 食品營養與保健生技學系
Pin-Jui Huang1, Cheng-Huang Shen1, Yeong-Chin Jou1,2, Chang-Te Lin1, Ming-Chin Cheng1
1Divisions of Urology, Department of Surgery, Chia-Yi Christian Hospital
2Department of Food Nutrition and Health Biotechnology, Asia University
Though angiomyolipoma (AML) is a common disease as renal tumor, the subtype of epithelioid angiomyolipoma (EAML) remains rare nowadays. The EAML had been recognized as a more invasive subtype of AML and higher conversion ratio to malignancy. The treatment of late stage or metastatic stage of EAML was confirmed that mTOR inhibitor had good treatment outcome. However, the treatment in early stage of EAML remained confused. In this article, we sincerely report a case who was diagnosed as early stage of EAML, and underwent radical nephrectomy. The treatment of mTOR inhibitor was soon prescribed after operation, and the general outcome seemed to be promising. The literatures of epithelioid angiomyolipoma is also reviewed in this article.
This 64-year-old female with underlying hypertension and peptic ulcer disease had history of left angiomyolipoma s/p transarterial embolization (TAE) in 2006. Tracing back her history, she experienced trauma event in 2006, and left flank pain with hematuria was found afterward. Abdominal computed tomography (CT) showed left renal mass with size 4.0cm, and perirenal hematoma was noted. Renal angiomyolipoma was suspected. Transarterial embolization was performed. Unfortunately, she lost follow-up after discharge. Another episode of left flank pain kicked off in 2020/11. There was episode of gross hematuria. She went to our gastrointestinal outpatient department (OPD) for help, and sonography revealed huge left upper pole renal mass. Abdominal CT with contrast enhancement showed interval progressive change of left renal angiomyolipoma, 10.8cm, with irregular soft tissue component. Operation of radical nephrectomy was performed, and the postoperative course was smooth. The renal function was fair postoperatively. The final pathology showed epithelioid angiomyolipoma. mTOR inhibitor was soon be prescribed after operation. There was no recurrent sign in follow-up CT image and any discomfort.
Discussion:
The treatment criteria of epithelioid angiomyolipoma were under debate for decades. EAML of the kidney is a specific type of renal AML with malignant potentials, where around 22% of the patients present with invasion or metastasis. The standard treatment was partial nephrectomy or radical nephrectomy, depending on the tumor size and location, and either the need of renal preserving. In 2020 NCCN guideline, mTOR inhibitor was suggested to be prescribed in case with recurrent or metastatic EAML. In Sanfillippo et al, mTOR inhibitor had better outcome and longer progression free survival (PFS) compared with chemotherapy, and VEGFR inhibitors in advanced EMAL. However, there was little to be mentioned in case of early stage EAML. In Chuang et al, it was described as that higher mitotic activities indicate a greater metastatic potential, with radical nephrectomy as the treatment of choice, and mTOR inhibitors such as everolimus either as neoadjuvant or adjuvant targeted therapy can lead to a better clinical outcome. In our case, mTOR inhibitor was soon be prescribed after operation. There was no recurrent sign in CT image in the first six-month follow-up.
This case reminds us that mTOR inhibitor could ensure a lower local recurrence rate and metastatic rate in case of early stage of epithelioid angiomyolipoma. We suggest that the treatment of mTOR inhibitor should be taken into consideration in such case for its promising outcome.