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I D Cancer Center
I D Cancer Center
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  • Von Hippel Lindau Disease

Von Hippel–Lindau (VHL) Disease Treatment | ID Cancer Center

Von Hippel–Lindau (VHL) disease is a rare, inherited genetic condition that causes tumors and cysts to develop in multiple organs, including the brain, spinal cord, eyes, kidneys, pancreas, and adrenal glands. Some tumors are benign, while others can become cancerous, making early diagnosis and lifelong monitoring essential.

What Is Von Hippel–Lindau Disease?

VHL is caused by a mutation in the VHL tumor suppressor gene, leading to abnormal cell growth. Key features include:

  • Hemangioblastomas (blood vessel tumors) – brain, spinal cord, retina

  • Kidney tumors & cysts – including renal cell carcinoma

  • Pancreatic cysts & neuroendocrine tumors

  • Adrenal tumors (pheochromocytoma)

  • Endolymphatic sac tumors (inner ear)

Because it affects multiple organs, coordinated, multidisciplinary care is essential.

Common Symptoms of VHL Disease

Symptoms vary depending on the organ involved:

Neurological (Brain & Spinal Cord)

  • Headache

  • Balance problems

  • Weakness or numbness

  • Vision disturbance

Eye (Retinal Hemangioblastomas)

  • Blurred vision

  • Floaters

  • Vision loss (if untreated)

Kidney (RCC or Cysts)

  • Blood in urine

  • Flank pain

  • Abdominal lump

Adrenal Gland (Pheochromocytoma)

  • High blood pressure

  • Palpitations

  • Sweating and anxiety

Pancreas

  • Abdominal pain

  • Hormonal symptoms (if neuroendocrine tumor)

Because VHL tumors can grow silently, regular screening is crucial.

How Is Von Hippel–Lindau Disease Diagnosed?

At I D Cancer Center, diagnosis involves:

1. Detailed Clinical Evaluation

Family history, symptoms, and physical examination.

2. Genetic Testing

Confirms VHL gene mutation.

3. Advanced Imaging

  • MRI of brain & spine

  • MRI abdomen for pancreas, kidney, adrenal glands

  • CT scan when required

  • Ultrasound for rapid tumor screening

4. Eye Examination

By a retinal specialist to detect early hemangioblastomas.

Early detection of tumors significantly improves outcomes.

Treatment Options for VHL Disease at I D Cancer Center

Treatment depends on tumor type, size, and location. Our multidisciplinary team includes oncology, neurology, urology, endocrinology, and radiology specialists.

1. Targeted Therapy (HIF-2α Inhibitors)

A major breakthrough in VHL treatment.

Belzutifan (HIF-2α inhibitor) effectively treats:

  • VHL-associated kidney tumors

  • Pancreatic neuroendocrine tumors

  • CNS hemangioblastomas

It reduces the need for frequent surgeries.

2. Surgery

Surgery may be required for:

  • Symptomatic or large brain/spine tumors

  • Renal cell carcinoma

  • Adrenal tumors (pheochromocytoma)

  • Pancreatic neuroendocrine tumors

Minimally invasive techniques help preserve organ function.

3. Advanced Radiotherapy (IMRT / SRS / SBRT / VMAT)

Used for:

  • Brain or spine hemangioblastomas

  • Renal cell carcinoma (select cases)

  • Non-operable tumors

At I D Cancer Center, precision treatments like SRS (Stereotactic Radiosurgery) and SBRT (Stereotactic Body Radiotherapy) offer excellent tumor control with minimal side effects.

4. Medical Management

Especially important for:

  • High blood pressure due to pheochromocytoma

  • Hormonal syndromes

  • Pain management