Von Hippel-Lindau (VHL) Disease

Definition

Von Hippel-Lindau (VHL) disease is an autosomal dominant tumor syndrome characterized by the development of benign and malignant tumors in multiple organs.

Symptoms

  • Central nervous system hemangioblastomas
  • Retinal angiomas
  • Kidney carcinoma
  • Pheochromocytoma

Reasons

Mutation in the VHL tumor suppressor gene.

Diagnosis

Clinical criteria, genetic testing, regular screening (MRI, ultrasound).

Treatment

Surgery for tumors, radiosurgery, regular follow-up.

Which Polyclinic Should You Apply To?

Genetics Polyclinic or Oncology Polyclinic

What to Do in an Emergency?

In case of neurological deficit due to brain tumor or pheochromocytoma crisis, emergency department should be consulted.

This page is for general information purposes only.