Von Hippel Lindau syndrome (VHL)

Hi all. Don't know if this is the right place for this - did a search, which brought up nothing.

Diagnosed with this 8 years ago; shan't bore you with the detail, but this is truly a genetic cancer nightmare. My father died of it aged 42 and his mother at the age of 36. It is only the wonders of modern surgery, radiology and chemistry that allows me to write this at the age of 58.

Currently (starting at the top!)

Haemangioblastoma inside the cervical spinal cord. They won't touch this until they have to.

Haemangioblastomas in both eyes. These are lasered as they arise.

Neuroendocrine tumours in the head of the Pancreas; awaiting biopsy results to see what can be done, if anything.

Phaeochromocytomas in both adrenal glands; both surgically removed with their associated glands.

Kidneys - multiple bilateral cysts, pre cancerous for RCC. Awaiting surgery.

I feel that I've managed to keep The Reaper skulking in the bushes for most of the time - just starting to get worried that he's making ground.

My thoughts are with all on here, and their loved ones, who have little hope. My experience, and my message - accept nothing, challenge all their decisions, push all the way. Never tolerate silence as an answer.

Richard.

Parents
  • Hi Richard, hope you are doing well. I have only signed up here. I have VHL so I can see what you are going through it’s not an easy disease to deal with. I wondered if you have heard of VHL patient having a lesion in the right inner ear on the inferior vestibular nerve I have been diagnosed with this I am concerned they mis diagnosing me and really it’s an Endolymphatic sac tumor but the scan report says no evidence of an Endolymphatic sac tumor. Have you or know anyone that had ended up with a VHL lesion on their right vestibular nerve? 

Reply
  • Hi Richard, hope you are doing well. I have only signed up here. I have VHL so I can see what you are going through it’s not an easy disease to deal with. I wondered if you have heard of VHL patient having a lesion in the right inner ear on the inferior vestibular nerve I have been diagnosed with this I am concerned they mis diagnosing me and really it’s an Endolymphatic sac tumor but the scan report says no evidence of an Endolymphatic sac tumor. Have you or know anyone that had ended up with a VHL lesion on their right vestibular nerve? 

Children
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