Systemic cystic angiomatosis in a woman with hematuria and splenomegaly.

Cutaneous hemangiomas are frequently found on routine clinical examination and generally are not important. But when internal organs are involved, these vascular tumours assume greater importance because of associated morbidity. A case is presented of multiple organ involvement by angiomas in a woman with a history of flank hemangioma who was investigated for hematuria and splenomegaly. She subsequently underwent splenectomy. The uncommon systemic syndromes characterized by vascular tumours are discussed, and the case reported is appropriately classified. The clinical picture, pathologic features and investigations appropriate for systemic cystic angiomatosis are briefly reviewed. The authors recommend conservative management when there is splenic involvement unless the size of the spleen makes its rupture likely or when there are hematologic complications.