Von Hippel-Lindau disease - bilateral pheochromocytomas

Discussion:

This case illustrates bilateral pheochromocytomas (left confirmed on histology) and a small presumed pancreatic neuroendocrine tumor in the setting of Von Hippel-Lindau disease

The presentation scan shows bilateral hypervascular adrenal lesions, which at this age group, almost invariably represents phaeocromocytomas (c.f. adrenal carcinoma will nor present bilaterally and metastases are very uncommon at this epidemiology). The 10% rule of thumb for phaeocromocytomas remind us that they can be bilateral, but also that they can bd a manifestation of an underlying genetic condition.  

 

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