[Unusual presentations of angiomyolipoma]

Angiomyolipoma is an uncommon benign tumor of the kidney. The tumor is composed of fat, smooth muscle, and blood vessels. The same pathological entity can appear in two clinically different forms, with or without tuberous sclerosis. We present two cases of renal angiomyolipomas with unusual manifestations. One was associated with tuberous sclerosis and both had the presentation of acute abdomen. Case one presented with epilepsy, angiofibroma, subungual fibroma, periventricular calcification, and bilateral renal angiomyolipomas. Tuberous sclerosis is characterized by these findings. Both cases had spontaneous hemorrhage with hypovolemic shock. Massive hemorrhage resulting in shock is uncommon and the incidence has been estimated to be about 10 per cent. In fact, many angiomyolipomas are clinically occult. The size of the tumor correlates well with the presence or absence of symptoms which include microhematuria, flank pain, hypertension and urinary tract infection. Abdominal CT is the preferred modality for diagnosis of angiomyolipoma. The most important finding is the presence of an intrarenal tumor with fat component which is recognized as a relative low density on CT. Our patients were hospitalized under the impression of angiomyolipoma after the CT study. In addition, the CT defined either the size of the tumor or the extension of the hemorrhage. Although many believe that renal angiography is not sufficient by itself to establish the diagnosis of angiomyolipoma, occasionally it is mandatory in the management of the tumor. The management is decided by two factors, the size of tumor and the clinical presentation. The attitude of management should include conservative treatment with regular follow-up, selective arterial embolization, enucleation, and partial or total nephrectomy.(ABSTRACT TRUNCATED AT 250 WORDS)