[Renal angiomyolipomatosis and pulmonary lymphangiomyomatosis. Its relationship with Bourneville syndrome].
BACKGROUND
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.


METHODS
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.


RESULTS
[corrected] 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.
Ponce Díaz-Reixa
J
J
Servicio de Urología, Hospital Juan Canalejo, A Coruña. ponce@canalejo.org
Barbagelata López
A
A
Romero Selas
E
E
Marcos Rodríguez
P
P
Sánchez Rodríguez-Losada
J
J
Alvarez Castelo
L
L
Fernández Rosado
E
E
González Martín
M
M
D015746:Abdominal Pain / Q000209:etiology; D000328:Adult; D018207:Angiomyolipoma / Q000000981:diagnostic imaging / Q000235:genetics* / Q000601:surgery / Q000628:therapy; D004322:Drainage; D004621:Embolization, Therapeutic; D004830:Epilepsy, Tonic-Clonic / Q000209:etiology; D005260:Female; D006801:Humans; D007680:Kidney Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics* / Q000601:surgery / Q000628:therapy; D008175:Lung Neoplasms / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics*; D018192:Lymphangioleiomyomatosis / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics*; D008207:Lymphatic Metastasis; D008875:Middle Aged; D009392:Nephrectomy; D011030:Pneumothorax / Q000209:etiology / Q000601:surgery; D012008:Recurrence; D014057:Tomography, X-Ray Computed; D014402:Tuberous Sclerosis / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*