Neoplasm associated with von Hippel-Lindau disease

A neoplasm associated with von Hippel-Lindau disease refers to any of the benign or malignant tumors and cysts that arise in patients with von Hippel-Lindau disease, a rare autosomal dominant hereditary cancer predisposition syndrome It predominantly affects adults and young adults across multiple organ systems, most commonly manifesting as central nervous system and retinal hemangioblastomas, clear cell renal cell carcinomas, pancreatic neuroendocrine tumors, and pheochromocytomas Pathophysiolo...

A neoplasm associated with von Hippel-Lindau disease refers to any of the benign or malignant tumors and cysts that arise in patients with von Hippel-Lindau disease, a rare autosomal dominant hereditary cancer predisposition syndrome

It predominantly affects adults and young adults across multiple organ systems, most commonly manifesting as central nervous system and retinal hemangioblastomas, clear cell renal cell carcinomas, pancreatic neuroendocrine tumors, and pheochromocytomas Pathophysiologically, germline inactivation of the VHL tumor suppressor gene impairs the degradation of hypoxia-inducible factors, notably HIF-2α, leading to constitutive pseudohypoxic signaling, overexpression of angiogenic growth factors, and hy...

Clinically, these neoplasms are characteristically multifocal, bilateral, and recurrent, often requiring specialized systemic management or nephron- and organ-sparing interventions

Clinically, these neoplasms are characteristically multifocal, bilateral, and recurrent, often requiring specialized systemic management or nephron- and organ-sparing interventions