Large aneurysm in renal angiomyolipoma causing life-threatening retroperitoneal hemorrhage

Large aneurysms occurring in renal angiomyolipomas (AML) may be encountered. When present, they have a higher propensity to hemorrhage and cause catastrophic intratumoral and perirenal hemorrhages. They also tend to occur more in tuberous sclerosis-associated AML rather than in the sporadic form. A 22-year-old female presented with sudden onset of acute right abdominal pain, hematuria and fainting. At evaluation in the emergency department, the patient was found to be in hemorrhagic shock with hemoglobin of 4 g/dl and low blood pressure of 60/40 mmHg. Fullness and tenderness of the right abdominal flank was also noted. Contrast enhanced CT scan of the abdomen showed a large fatty mass of the right kidney with a surrounding perirenal hematoma. A 4.0 cm wide focus of contrast was seen within the mass highly suspicious of an aneurysm. A subsequent DSA angiogram of the right kidney confirmed the presence of the aneurysm within the mass as the cause of the hemorrhage. The patient underwent successful emergency coil and particle embolization of the aneurysm and mass with control of the bleeding. The patient was diagnosed with tuberous sclerosis syndrome with further clinical findings of cutaneous adenoma sebaceum and lung cystic changes.