Dopamine-secreting adrenal ganglioneuroma presenting with paroxysmal hypertension attacks

Adrenal ganglioneuromas are rare tumors originating from the neural crest tissue of the sympathetic nervous system. The clinical presentation for most patients is asymptomatic and most of these tumors are hormone silent. We report a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient. A 46-year-old woman was admitted to our hospital with a 2-month history of right flank pain and a-year history of paroxysmal hypertensive attacks associated with headaches, palpitations, nervousness and sweating. Abdominal CT and MRI revealed a solid round tumor approximately 4 cm in diameter on the upper pole of the right kidney. Urinary levels of dopamine and homovanillic acid were slightly elevated, although urinary levels of metanephrine and normetanephrine were suppressed. The urinary levels of epinephrine, norepinephrine and vanillylmandelic acid were within normal limits. Right adrenalectomy was performed for treatment purposes. Histological diagnosis of the tumor was ganglioneuroma originating from the adrenal medulla. In conclusion, this is a case of dopamine-secreting adrenal ganglioneuroma associated with paroxysmal hypertensive attacks in an adult patient. (author)