Composite pheochromocytoma-ganglioneuroma in patient with neurofibromatosis type 1

Neurofibromatosis type 1 (NF-1) or Von Recklinhousen's disease is a multisystem disease of autosomal dominant inheritance that primarily affects the skin and nervous system. Diagnosis is clinical and can be confirmed by genetic testing, but technically complex and does not predict the occurrence of complications, so performing it routinely is not indicated. Neurofibromatosis type 1 is associated with various endocrine diseases, one of which pheochromocytoma. The compounds pheochromocytomas are rare tumours which have also been associated with this syndrome. Pheochromocytomas are associated with tumours with the same embryonic origin, the ganglioneuromas being the most frequent. The prevalence of pheochromocytoma and ganglioneuroma may be increased in patients with NF-1 and this has been associated with more aggressive tumours, so this article stresses the importance of evaluating these patients to avoid complications related to the tumour if there was not an early diagnosis. We present the case of a pheochromocytoma compound with ganglioneuroma in an asymptomatic patient affected from NF-1 and the most relevant aspects of this tumour are reviewed.