Background
Ganglioneuromas are rare benign tumours originating from the neural crest tissue.[1] Ganglioneuromas are slow-growing usually asymptomatic tumours.[1,2] 30% of these tumours secrete catecholamines, but exclusively dopamine-secreting ganglioneuromas, besides being extremely rare, lack the classical presentation of sympathetic activation.[1,2,3] We report the case and perioperative anaesthetic management of a 41-year-old man with a dopamine-secreting ganglioneuroma presenting with paroxysmal hypertension.

Case report
A 41-year-old man was diagnosed with an exclusively dopamine-secreting retroperitoneal ganglioneuroma during a workup for a 6-month history of hypertensive crisis. After consultation with a multidisciplinary team, the patient was scheduled for en bloc resection under general balanced anaesthesia. Preoperative control of arterial pressure was achieved with amlodipine/valsartan, phenoxybenzamine and propranolol. After tumour ligation, profound hypotension developed and was successfully treated with dopamine, noradrenaline and methylene blue. The patient was extubated at the end of the surgery. Postoperative period was uneventful, the patient being discharged at 5th PO day. To the current day, the patient remains asymptomatic with normal blood pressure and dopamine urinary levels.

Discussion
Dopamine-secreting ganglioneuromas presenting with hypertension are rare entities, with only 2 cases reported in the literature.[1,2] There is no consistent approach to these rare tumours. 
Similar to noradrenaline/adrenaline secreting tumours, we considered the primary goal the provision of stable hemodynamics during all perioperative period.

References
[1] Saudi Med J 2008;29:122-125
[2] Koch C. et al. Endocrine-Related Cancer 2003;10:99-107
[3] JBR–BTR 2014;97:109-112

Learning points
Facing a cathecolamine-secreting tumour presenting with hypertension, dopamine excess should be investigated.
We suggest that management of dopamine-secreting ganglioneuromas presenting with hypertension be similar to that of noradrenaline/adrenaline-secreting ones.


05 / Dopamine-secreting ganglioneuroma: anaesthetic management of a rare entity with an atypical presentation