ANGIOMYOLIPOMA – VARIED PRESENTATIONS – A CASE SERIES

Abstract

Renal angiomyolipoma is a benign mesenchymal tumour composed of fat cells, smooth muscle cells, and thick-walled blood vessels. These tumors can occur sporadically or as part of genetic syndromes: most commonly tuberous sclerosis complex. Here we present three cases of Renal angiomyolipoma(AML) in different scenarios - presenting with clinical features like ank pain, abdominal mass or perirenal haematoma with hypovolemic shock. All three cases were evaluated by contrast enhanced CT & diagnosed as renal angiomyolipoma. Nephron sparing surgery - Partial nephrectomy was done in case 1. Case 2 underwent angiography with non- selective angioembolisation preoperatively followed by simple nephrectomy. Case 3, after initial stabilization and evaluation found to be a case of Wunderlich syndrome and simple nephrectomy done. Post intervention period was uneventful for all 3 cases. In the 6-month follow-up, all three patients were doing well with normal renal function and no recurrence in case of partial nephrectomy. Embolisation is the emergency treatment of choice for bleeding angiomyolipoma. Whenever possible, a nephron sparing approach by either transarterial embolisation or partial nephrectomy is to be considered. In cases of angiomyolipoma of both kidneys or in a solitary functioning kidney, renal preservation is mandatory in order to avoid need for renal replacement therapy. Also, recently approved drug Everolimus may be considered for patients not suitable for surgery particularly in tumour seen with tuberous sclerosis