Concurrent hepatic and ruptured renal angiomyolipoma in tuberous sclerosis complex.

Wunderlich's syndrome with hepatic angiomyolipoma in tuberous sclerosis.

We present a patient of tuberous sclerosis complex with bilateral renal angiomyolipoma and hepatic angiomyolipoma with spontaneous retroperitoneal hemorrhage from right angiomyolipoma (Wunderlich's syndrome). Nephron preserving approach was tried but the right kidney could not be salvaged. Hepatic angiomyolipoma was managed conservatively. Wunderlich's syndrome has been found in around 10% of c...

Pulmonary Lymphangiomyomatosis Associated with Renal and Hepatic Angiomyolipoma Mass in a Patient with Tuberous Sclerosis Complex

Intrarenal aneurysms in the tuberous sclerosis complex

The tuberous sclerosis complex (TSC, OMIM 191100) is a rare autosomal dominant syndrome of high penetrance characterized by hamartoma in multiple organs, namely brain, kidneys, heart, skin, eyes and lungs [1]. The classic clinical triad includes seizures, mental retardation and cutaneous adenoma sebacea. Besides common vascular abnormalities observed in angiomyolipoma, TSC can be associated wit...

Hepatic manifestations of tuberous sclerosis complex: a genotypic and phenotypic analysis.

Hepatic manifestations of tuberous sclerosis complex: a genotypic and phenotypic analysis. A retrospective review of the clinical records and radiological images of 205 patients with tuberous sclerosis complex (TSC) was performed to evaluate the prevalence and progression of hepatic lesions; examine the association of hepatic phenotype with genotype, age, and gender; and investigate the relatio...

Tuberous sclerosis complex-associated kidney angiomyolipoma: from contemplation to action.

2012-SAP029 ERA-EDTACongress 16. Balligand JL, Pirson Y, Squifflet JP et al. Outcome of patients with tuberous sclerosis after renal transplantation. Transplantation 1990; 49: 515–518 17. van Slegtenhorst M, de Hoogt R, Hermans C et al. Identification of the tuberous sclerosis gene TSC1 on chromosome 9q34. Science 1997; 277: 805–808 18. European Chromosome 16 Tuberous Sclerosis Consortium. Iden...