Ganglioneuroblastoma is a rare neoplasm of adults, containing both primitive neuroblastomatous and mature ganglioneuromatous elements of sympathetic cell origin. We describe an adult patient with a ganglioneuroblastoma exclusively secreting dopamine.
A 41 year old lady represented 6 days post laparoscopic excision of endometriosis with a 2 day history of right sided abdominal pain. Only on direct questioning did she admit to a vague 2 month history of occasional episodes of flushing, sweating and palpitations. Abdominal examination revealed a tender mass in her right upper quadrant. She was consistently normotensive. Ultrasound examination suggested an adrenal mass. Multiple 24hr urine collections for catecholamines showed elevated dopamine levels 6290-7959 nmol/24hr (ref. range 650-3270) with normal adrenaline and noradrenaline levels. Abdominal CT and subsequent MIBG scan confirmed an extensive neuroendocrine tumour in her right upper quadrant. Further imaging did not show tumour deposits elsewhere. She underwent surgery to resect a melon sized, non-infiltrating, retroperitoneal, right paraaortic tumour in its entirety. Histology revealed a ganglioneuroblastoma in which the ganglion cell component is immature and the S100 positive spindle cells represent schwannomian differentiation. She remains extremely well 17 months post surgery with normal MIBG and Indium Octreotide scans and normal serial urine catecholamines estimations.
This case describes an extremely rare purely dopamine secreting ganglioneuroblastoma which was resected with no signs of recurrence to date.
03 - 04 Dec 2001
Society for Endocrinology