Von Hippel-Lindau Syndrome

VHL is a rare tumor predisposition syndrome characterized by tumors such as hemangioblastoma, renal cell carcinoma, and pheochromocytoma. Although patients are often diagnosed within the 2nd to 3rd decade of life, patients may present with tumors during childhood. Patients are diagnosed based on family history, clinical findings, and genetic testing. Long-term follow-up and screening is necessary due to the high likelihood of tumors throughout the body.