Abstract

PONCE DIAZ-REIXA, J. et al. Renal angiomyolipomatosis and pulmonary lymphangiomyomatosis: It's relationship with bourneville syndrome. Actas Urol Esp [online]. 2006, vol.30, n.4, pp.386-393. ISSN 0210-4806.

Introduction: Renal angiomyolipomas are not very frequent in urologic activities. Pulmonary lymphangioleiomyomatosis is a rare disease that occurs mainly in women of reproductive age. Tuberous sclerosis shows lesions to different organs, including these both entities. Material and method: We retrospectively analyze patients treated in our center. Three most representatives cases are reported. An indexed literature review is done, in order to establish a clinical protocol to manage these kinds of patients. Resultados: Between 1990 and 2004, eight patients have been attended with an angiomyolipoma renal diagnostic. Five women (62%) and three men (38%). Medium age is 52,3y (29-69). In 3 of them (all women), it was also done a diagnostic of pulmonary lymphangioleiomyomatosis. Conclusions: 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.

Keywords : Renal angiomyolipoma; Pulmonary lymphangioleiomyomatosis; Bourneville syndrome; Tuberous sclerosis complex.

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