Statistics from Altmetric.com
Tuberous sclerosis is a systemic disorder characterised by benign hamartomatous tumours that involve multiple organ systems. Angiomyolipomas are found in about two thirds of the kidneys of patients with tuberous sclerosis at necropsy.1
A 29 year old woman with tuberous sclerosis presented with diffuse abdominal pain, gross haematuria, and hypotension. At the age of 22 the patient had been admitted to our hospital with acute upper abdominal pain and gross haematuria. Physical examination had revealed bilateral massive abdominal masses with no tenderness. Computed tomography had confirmed the presence of giant bilateral renal masses with the typical appearance of an angiomyolipoma, and a huge retroperitoneal haematoma extending to the pelvis and the left side of the abdomen (fig 1). After arterial embolisation, left radical nephrectomy was performed. The pathological diagnosis was angiomyolipoma without malignant findings, and the patient was discharged two weeks after the operation (fig 2).
During the latest admission, computed tomography of the abdomen showed a giant right renal angiomyolipoma (fig 3). An attempt for selective arterial embolisation failed, and right radical nephrectomy was performed as the patient was becoming haemodynamically unstable. The patient has been started on haemodialysis, and renal transplantation is planned.
Kidneys can be involved in three different ways in tuberous sclerosis: angiomyolipomas, cystic disease, and renal cell carcinomas.1 The typical angiomyolipoma is a mixture of smooth muscle, fat, and abnormal blood vessels, the relative proportions of which vary widely.1 Although angiomyolipomas were previously considered to be hamartomas, they have been recently recognised as clonal neoplasms, and it has been proposed that the perivascular epithelioid cell is the progenitor cell.2 From the clinical point of view, angiomyolipomas are usually silent, but they can rarely cause life threatening haemorrhage. Patients with tuberous sclerosis and angiomyolipomas greater than 4 cm have a high risk of developing symptoms and may require surgery.1 Because of its sensitivity in detecting small amounts of fat, computed tomography is the imaging modality of choice, although the typical appearance may be altered in predominantly non-fatty angiomyolipomas or in tumours in which haemorrhage has occurred. Long term follow up results of patients with angiomyolipomas treated with selective arterial embolisation have been reported recently.3 Selective arterial embolisation seems to be ideal in the case of acute haemorrhage in selected patients with renal angiomyolipoma but its indication, safety, and efficacy require further evaluation.
If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.