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Renal tumors in the Phakomatosis disease, what to note: two case report
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Abstract
SUMMARY
Phakomatosis are a group of neurocutaneous disorders characterised by involvement of structures that arise from the embryonic ectoderm , consist of central nervous system, skin ,eyes and others: kidney, heart, lung… In this article we focus on the incidence of renal tumors in Tuberous sclerosis, one of the common diseases in Phakomatosis. Tuberous sclerosis is a
rare neurocutaneous disorder (phakomatosis) characterized by the development of multiple benign tumours in various organs, including the kidneys. Tuberous sclerosis complex has several renal manifestations including angiomyolipomas (AML) and renal epithelial neoplasms. Angiomyolipomas is found in 40% of patients with tuberous sclerosis, and its most common
complication is ruptures due to aneurysms. We present two cases was diagnosed with bilateral angiomyolipomas in tuberculosis and emphasizes the importance of the diagnosis and choice of appropriate treatment for each patient.
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Keywords
Phakomatosis, renal tumor
References
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