Tuberous sclerosis complex is a multiorgan syndrome manifesting with several benign and malignant tumors. Complications arising from renal abnormalities are a leading cause of death in patients with tuberous sclerosis complex. Renal cell carcinoma is relatively uncommon, occurring in 2%-4% of patients with tuberous sclerosis complex syndrome, but nonetheless can significantly contribute to morbidity and mortality. Extrarenal manifestations of tuberous sclerosis complex, including within the chest, abdomen and central nervous system, aid in diagnosis. Pathogenesis and management are also discussed, including the importance of the types of renal masses found in these patients.
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http://dx.doi.org/10.1016/j.acra.2020.12.019 | DOI Listing |
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