By what age will a patient with VHL disease likely develop bilateral renal cell carcinoma?

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Von Hippel-Lindau (VHL) disease is a genetic condition characterized by the development of various tumors, including bilateral renal cell carcinoma (RCC). Individuals with VHL have mutations in the VHL gene, which plays a critical role in regulating hypoxia-inducible factors, and as a result, they are at increased risk of developing several types of tumors, particularly in the kidneys.

Patients with VHL disease generally begin to develop renal cell carcinoma at a relatively young age compared to the general population. While there is variability, studies indicate that the onset of bilateral RCC in VHL patients commonly occurs around the age of 50. However, significant numbers of patients may actually present with renal tumors earlier than this, with findings showing that many individuals may have developed RCC by their late 30s or early 40s.

The likelihood of developing bilateral RCC by age 60 is consistent with the disease's known progression, as studies show that most affected individuals will have manifestations of RCC by this time. For those with VHL, surveillance and early intervention are crucial for managing these tumors, as early detection can significantly impact outcomes.

In summary, the association of VHL disease with the development of bilateral renal cell carcinoma typically leads to diagnosis around the

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