[Angiomyolipoma of the kidneys as a rare cause of retroperitoneal hemorrhage. Two case reports with tuberous sclerosis Bourneville-Pringle].
Tuberous sclerosis (Bourneville-Pringle-disease, TSC) is an autosomal dominant disorder characterized by seizures, mental retardation and hamartomatous tumours in multiple organs, including subependymal giant cell astrocytomas, cardiac rhabdomyomas and renal angiomyolipomas. Recent population-based studies suggest a prevalence of 1 case per 25,000 individuals. Renal angiomyolipomas, which may be found sporadically or associated with TSC, become evident as an acute retroperitoneal haemorrhage or by symptoms of a flank mass. Ultrasound and computed tomography provide clear evidence of lipomatous formation while, in rare instances, angiography can demonstrate the existence of multiple vascular tumour compartments. In view of two cases which were admitted with the clinical picture of an acute abdomen on the basis of retroperitoneal haemorrhage, the therapeutic strategies for TSC patients with renal angiomyolipomas are discussed, paying regard to the literature in this field.
May
M
M
Urologische Klinik, CTK Cottbus, Akademisches Lehrkrankenhaus der Charité zu Berlin. M.May@ctk.de
Seehafer
M
M
Helke
C
C
Stosiek
P
P
Ehlers
C
C
Hoschke
B
B
D000006:Abdomen, Acute / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D000328:Adult; D018207:Angiomyolipoma / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D003937:Diagnosis, Differential; D005260:Female; D006470:Hemorrhage / Q000209:etiology* / Q000473:pathology / Q000601:surgery; D006801:Humans; D007668:Kidney / Q000473:pathology; D007680:Kidney Neoplasms / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D008297:Male; D009378:Neoplasms, Multiple Primary / Q000150:complications* / Q000235:genetics / Q000473:pathology / Q000601:surgery; D009392:Nephrectomy; D012187:Retroperitoneal Space; D012422:Rupture, Spontaneous; D014402:Tuberous Sclerosis / Q000150:complications* / Q000235:genetics / Q000473:pathology