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Tuberous Sclerosis Complex and the kidneys: what nephrologists need to know
Aline Grosskopf Monich1,2, John J Bissler3,4,5, Fellype Carvalho Barreto1,6
1Universidade Federal do Paraná, Departamento de Clínica Médica, Programa de Pós-Graduação em Medicina Interna e Ciências da Saúde, Curitiba, PR, Brazil.
Jornal Brasileiro De Nefrologia
|July 11, 2024
Summary
Tuberous sclerosis complex (TSC) is a genetic disorder causing tumors in various organs. mTOR inhibitors are now the main treatment for kidney lesions in TSC, shifting away from surgery.
Area of Science:
- Genetics and Molecular Biology
- Oncology
- Nephrology
Background:
- Tuberous sclerosis complex (TSC) is an autosomal dominant disorder.
- It results from TSC1 or TSC2 gene mutations, leading to mTOR pathway hyperactivation.
- This pathway dysregulation drives abnormal cell growth and tumor formation.
Purpose of the Study:
- To review TSC's clinical features, focusing on kidney involvement.
- To discuss the pathophysiology of TSC-related kidney lesions.
- To highlight recent therapeutic advances and future directions for TSC kidney manifestations.
Main Methods:
- Literature review of clinical characteristics and therapeutic strategies for TSC.
- Analysis of the role of the mTOR pathway in TSC pathogenesis.
- Synthesis of current research on kidney lesion management in TSC.
Main Results:
- TSC causes hamartomas in multiple organs, including the kidneys.
- Kidney lesions in TSC include cysts, renal cell carcinoma, and angiomyolipomas.
- mTOR inhibitors represent a significant therapeutic advancement for TSC kidney disease.
Conclusions:
- The therapeutic landscape for TSC kidney lesions has shifted towards medical management with mTOR inhibitors.
- Surgery is now reserved for severe, treatment-resistant complications.
- Further research is needed to optimize long-term management and explore novel therapies.
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