What type of renal tumor is associated with von Hippel-Lindau syndrome?

What type of renal tumor is associated with von Hippel-Lindau syndrome?

• A. Renal cell carcinoma
• B. Wilms tumor
• C. Transitional cell carcinoma
• D. Clear cell carcinoma

Answer: (A)

Von Hippel-Lindau (VHL) syndrome is a genetic disorder characterized by the development of various tumors and cysts in multiple organs, predominantly affecting the kidneys. The most notable renal tumor associated with this syndrome is renal cell carcinoma, specifically the clear cell type, which is often linked to alterations in the VHL tumor suppressor gene. Individuals with VHL syndrome are at an increased risk of not only renal cell carcinoma but also pheochromocytomas, hemangioblastomas of the central nervous system, and pancreatic neuroendocrine tumors. The clear cell subtype of renal cell carcinoma is particularly prominent in VHL, as loss of the VHL gene function leads to the disruption of pathways that regulate angiogenesis and cell proliferation, resulting in the hallmark characteristics of clear cell carcinoma. Other tumor types mentioned, such as Wilms tumor, transitional cell carcinoma, and different cell types of renal cell carcinoma, do not have a direct association with VHL syndrome. Therefore, when considering the link between VHL syndrome and renal tumors, renal cell carcinoma, specifically the clear cell variant, is the most commonly recognized entity.

Von Hippel-Lindau (VHL) syndrome is a genetic disorder characterized by the development of various tumors and cysts in multiple organs, predominantly affecting the kidneys. The most notable renal tumor associated with this syndrome is renal cell carcinoma, specifically the clear cell type, which is often linked to alterations in the VHL tumor suppressor gene.

Individuals with VHL syndrome are at an increased risk of not only renal cell carcinoma but also pheochromocytomas, hemangioblastomas of the central nervous system, and pancreatic neuroendocrine tumors. The clear cell subtype of renal cell carcinoma is particularly prominent in VHL, as loss of the VHL gene function leads to the disruption of pathways that regulate angiogenesis and cell proliferation, resulting in the hallmark characteristics of clear cell carcinoma.

Other tumor types mentioned, such as Wilms tumor, transitional cell carcinoma, and different cell types of renal cell carcinoma, do not have a direct association with VHL syndrome. Therefore, when considering the link between VHL syndrome and renal tumors, renal cell carcinoma, specifically the clear cell variant, is the most commonly recognized entity.