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Neuroblastoma

 

 

Associated Syndromes

These syndromes are secondary to neuroblastoma (NBL):

 

Opsoclonus-myoclonus:

  • Acute cerebellar ataxia
  • Rapid random eye movements
  • May present years prior to the neuroblastoma itself
  • Autoimmune disorder (caused by IgG and IgM antibodies)
  • Associated with thoracic, localized NBL
  • Good disease free survival
  • 80% have permanent neurological sequelae

There is a very helpful video of opsoclonus-myoclonus posted on You Tube by a mother whose child has this syndrome.  She writes:

"My son Jonah aged 5 with a very clear shot of his dancing eyes (opsoclonus myoclonus).  I shot this video over the top of the hospital TV and you can see when he spots I am there. Background info: Jonah suddenly stopped walking last October and then his arms went all wobbly and then his eyes went. It took 6 weeks for the hospital in London to find the tumour. He has had a resection (operation to remove the tumour) but unfortunately the tumour is growing again. He has dexamethasone steroids IV through his port each month but other than that has had no other treatment. As you can see from the video, he is still a very happy little boy regardless of all the problems he has!!"

 

Pepper Syndrome:

  • Massive involvement of liver with metastatic disease
  • May have respiratory distress

 

Horner's Syndrome:

  • Unilateral ptosis, myosis, anhydrosis
  • Associated with thoracic and cervical tumors
  • Does not resolve with tumor resection

 

Hutchinson Syndrome:

  • Limping and irritability in young child
  • Associated with bone and bone marrow metastases

 

Kerner-Morrison Syndrome:

  • Intractable secretory diarrhea
  • Due to vasointestinal peptide (VIP) secretion
  • Generally associated with favorable tumors

 

 

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