Abstract
Pheochromocytomas and functional paragangliomas are rare neuroendocrine tumours with very variable clinical picture which may remain undiagnosed until death or until they cause life-threatening complications. Due to genetic heterogeneity, these tumours may differ in the age of presentation, clinical and biochemical phenotype and susceptibility to develop metastases.
The most important part of correct diagnosis is the awareness of the tumour. Referring the patient to the specialized centres should be taken into consideration when- ever possible.
Tis is crucial not only for the biochemical and morphological diagnosis but also for the best surgical management and subsequent follow-up.