The Paraganglioma of the adrenal medulla

• A type of malignant neuroendocrine tumor arising from a SNS paraganglia
• Involve nerve calls calledz chromaffin cells

Etiology

• 50% has Genetic Predisposition: highly heritable
  • younger = more likely to be genetic
  • but most patient lack FMHx
  • Genetic syndromes:
• degree of metastatic spread depends on genotype

Diagnosis

• Diagnosis:

DDx:

• Rule out other adrenal causes
  • Cushing: Dexamethasone Suppression Test
  • Primary aldosterone: Aldosterone and Renin
• Rule out renal causes
  • Electrolyte and sCr (60-100 umol/L)

Imaging

• Abdo CT IV Contrast
• Abdominal MRI
• MIBG

Treatment

• Genetic test and counciling
• Medication (pre-surgical management)
  • BP must be very well controlled prior to surgery since there is a risk of triggering a massive release of previously synthesized catecholamines when the tumour is handled → !Malignant HTN (220/130)
    • Alpha-1 Blocker (-”osin”) : for hypertension + Beta Blocker (”-prolol”): for tachycardia
      • Do not give beta blocker in isolation → unopposed vasoconstriction worsens hypertension
• Surgical removal of the adrenal gland