Renale Angiomyolipome assoziiert mit tuberöser Hirnsklerose als seltene Ursache einer abundanten retroperitonealen Blutung

Abstract

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.

A diagnostic dilemma: lupus nephritis or renal polyarteritis nodosa?

“…6 Although, PAN may cause peri-renal hematoma, 7 several other etiologies of Wunderlich's syndrome have been reported in the literature including renal cell carcinoma, angiomyolipoma, vasculitis, cystic renal diseases, and nephritis. [8][9][10][11] In conclusion, lupus nephritis and PAN may coexist together causing a diagnostic dilemma, and lupus nephritis may play a role in the pathogenesis of renal PAN. The overlapping presentation of renal lupus and PAN also makes the management of these patients extremely challenging due to ambiguous clinical picture, difficulty in obtaining follow-up kidney biopsies, and unclear prognosis.…”