Angiolipomatosis renal y linfangioleiomiomatosis, su relación con la enfermedad de Bourneville

Bourneville syndrome is not very frequent. Associated angiomyolipomas usually are multiple, bilateral, with tender to grow and require more interventions. Previous diagnostic let us prevent future complications with vigilance, selective arterial embolization and nephron-spare surgery. Tumour size and the presence of symptoms are more decisive to decide best therapy alternative. Young patients with pulmonary lymphangioleiomyomatosis should be advise against pregnancy and the use of preparations containing estrogens.