Von Hippel-Lindau syndrome (VHL) is an autosomal dominant hereditary disease characterized by a variety of benign/malignant tumors, mainly hemangioblastomas, renal, pancreatic, and hepatic alterations, as well as pheochromocytoma. The disease is associated with high morbidity and mortality and can affect multiple family members. Due to the potential for serious complications and psychosocial impacts, early diagnosis is crucial for monitoring tumor lesions, multidisciplinary treatment, and appropriate genetic counseling.
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