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
Primary renal epithelioid angiomyolipoma (EAML) is a rare subtype of renal angiomyolipoma (AML), which has a higher conversion ratio to malignancy. EAML also has high recurrent and metastatic potential. In this article, we sincerely introduce a case who was diagnosed as EAML, with multiple rapid recurrent episodes postoperatively, even under treatment of mTOR inhibitor. Liver and lung metastases were noted during follow-up. The literatures of epithelioid angiomyolipoma are also reviewed in this article.
This is a 65-year-old female had past history of liver cirrhosis. She was firstly diagnosed as left renal epithelioid angiomyolipoma (EAML) in 2008 after left radical nephrectomy. However, recurrent tumor was noticed in 2011/08 and 2012/01, and she received tumor excision and splenectomy consequentially. The final pathology was both EAML. Local recurrent tumor at upper and lower retroperitoneal space was noted in 2012/04. Self-paid mTOR inhibitor with Everolimus was prescribed. Stationary disease was noticed via abdominal computed tomograph (CT) during 2012/11-2013/02. However, progression of disease with tumor size up to 8cm and 14cm at upper and lower retroperitoneal space was noted via CT in 2013/07. Stationary disease was noted during follow-up from 2013/04-2015/12. The patient discontinued to use self-paid Everolimus due to economical consideration in 2015. In 2016/04, abdominal CT showed the upper retroperitoneal tumor with adhesion to stomach. At the same month, the patient suffered from tarry stool, and panendoscopy revealed protruding mass with superficial ulceration at stomach body, favored external invasion. Operation of partial gastrectomy and left D-colectomy with T-S colostomy was performed in 2016/04/25. The final pathology was EAML. Follow-up abdominal CT in 2016/06 revealed 6.7cm residual tumor at left iliac region, and multiple hepatic tumors were noted in 2016/10. Biopsy of liver tumor was performed, and the pathology showed EAML. Transaterial chemoembolization (TACE) was performed for two times in 2016/11 and 2017/03. However, One small lung nodule with size 0.8cm was noticed at left lower lung (LLL) via CT in 2017/05. The patient was admitted to our hospital in 2017/06 due to ileus. gastrointestinal bleeding and intra-abdominal infection were noticed during admission. Fluid resuscitation and inotropic agent did little help, and the patient was declared death on 2017/06/24.
Discussion:
Epithelioid angiomyolipoma could be invasive and highly metastatic. 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 and metastatic EAML. In Guo et al, 29% patients with EAML under mTOR inhibitor treatment after operation experienced rapid progression within six months. However, mTOR inhibitor seemed to be effective in this case, with progression free survival of 32 months. In Vicens et al and Zhu, it was reported that distant visceral metastasis could happen in liver and pancreas. In our case, the visceral metastases ware identified in both liver and lung, which represented the highly metastatic nature of EAML.
This case reminds us that epithelioid angiomyolipoma could be highly invasive but could be stabilized by therapy of mTOR inhibitor. Moreover, the potential of distant metastasis should be highly alerted. Regular examination of abdominal CT should be scheduled during follow-up.