Definition of Angiomyolipoma of the Kidney
Angiomyolipoma of the kidney is a rare benign renal tumor with a high fat content.
Epidemiology of Renal Angiomyolipoma
Prevalence:
The prevalence in autopsies is 0.3%, with ultrasound screening 0.1%. Women are much more frequently affected than men. Age peak 50–60 years.
Tuberous sclerosis:
45–80% of patients with tuberous sclerosis have (bilateral) asymptomatic angiomyolipomas. In tuberous sclerosis, there is an equal distribution between men and women, age peak 30 years.
Etiology and Pathology of Angiomyolipoma
Etiology:
Angiomyolipomas derive from perivascular epithelioid cells and grow probably hormone-dependent.
Gross Pathology of Angiomyolipoma:
Angiomyolipomas are grey-yellow lesions without a tumor capsule, round to oval. Sometimes, angiomyolipomas show multi-center growth with involvement of lymph nodes without metastatic potential.
Histology of Angiomyolipoma:
Mature fat cells, smooth muscle cells, (atypical) blood vessels, occasional mitoses.
Signs and Symptoms of Angiomyolipoma of the Kidney
- Flank pain
- Potentially life-threatening hemorrhage due to spontaneous rupture into the retroperitoneum (Wunderlich's syndrome). Pregnancy increases the risk of rupture.
Diagnosis of Renal Angiomyolipoma
Sonography of the Kidney:
Sonography of the kidneys shows an echogenic mass in the kidney, caused by the high fat content.
CT-Abdomen:
The fat content typically causes a hypodense mass in the kidney with -20 to -80 HU, this enables the differentiation to renal cell carcinoma [fig. CT of angiomyolipoma]. Calcifications are rare in renal angiomyolipoma.
| | Fig. CT abdomen of renal angiomyolipoma: visible are hypodense fatty portions of the kidney tumor. By courtesy, Dr. G. Antes, Kempten. |
MRI Abdomen:
MRI enables the reliable detection of fat, which is typical for angiomyolipoma, and allows the differentiation to a renal cell carcinoma.
Angiography:
Neovascularization, similar to renal cell carcinoma, are possible. Angiography has no role in the differential diagnosis of renal tumors.
Treatment of Renal Angiomyolipomas
Conservative Treatment of Angiomyolipomas:
Annual imaging controls are possible with angiomyolipomas less than 4 cm and mild symptoms. The annual growth rate is about 5%, in tuberous sclerosis up to 20%.
Partial Nephrectomy:
Partial nephrectomy is indicated in angiomyolipomas of >4 cm, severe symptoms or in tumors of uncertain diagnosis.
Selective embolization:
Selective embolization is a possible minimally invasive treatment option. Sometimes, a relapse is possible after embolization. Rarely, percutaneous drainage of necrosis is necessary.
Emergency nephrectomy:
Nephrectomy is necessary in rupture of angiomyolipoma with life-threatening bleeding.