Dopamine-secreting adrenal ganglioneuroma in a child: beware of intraoperative rebound hypertension

Catecholamine-secreting adrenal lipomatous ganglioneuroma: A case study

“…The second aspect of interest is that we identified high concentrations of catecholamines. Although ganglioneuromas are thought to have a low or no metabolic activity, several previous studies have reported high concentrations of catecholamines or MIBG uptake in intra- and extra-adrenal ganglioneuromas [10] , [11] , [12] , [13] . Specifically, Geoerger et al reported that 39% of ganglioneuromas (18 of 46 cases) were associated with higher concentrations of catecholamines and 57% (17 of 30 cases) were associated with detectable MIBG uptake [10] .…”

Case report of severe psychiatric sequelae in a 16-year-old female following resection of a purely dopamine-secreting ganglioneuroma

“…Ganglioneuromas are rare neural crest tumors that represent 6.4–20% of all neuroblastic tumors and have an overall reported incidence of 1 in 1,000,000 [1,[7], [8], [9]]. Many of these tumors are diagnosed when they grow large enough to affect surrounding structures, with the most common sites of origination being the adrenal medulla, organ of Zuckerkandl, or along the paravertebral sympathetic ganglia [1,9]. In one study, these tumors showed a predilection for growth within the thoracic cavity in 41.5% of cases, the abdominal cavity excluding adrenal origins 37.5% of the time, and 21% localized to the adrenal gland [5].…”

“…Few ganglioneuromas are hormonally active, a characteristic more often associated with neuroblastomas, pheochromocytomas, and paragangliomas [9]. Those that do exhibit metabolic activity usually secrete the catecholamines epinephrine and norepinephrine, either exclusively or in combination [11].…”

“…Ganglioneuromas can also secrete dopamine, either exclusively or in addition to epinephrine and norepinephrine. These are exceptionally rare as dopamine-secreting tumors are generally extra-adrenal pheochromocytomas and a review of the existing world literature highlights only 17 published cases and reports of dopamine producing ganglioneuromas to our knowledge [9,[14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29]]. Of these, only 5 had exclusive secretion of dopamine or had excessive amounts of its inactive metabolite HVA, whereas the remainder were found to also secrete epinephrine and norepinephrines or had detectable levels of their inactive metabolite, VMA [14,[24], [25], [26],29].…”

“…GNs may become symptomatic if large enough to be palpated or due to mass effect on surrounding structures. Unlike in NBs, GNs are infrequently hormonally active and therefore, rarely cause symptoms due to the secretion of catecholamines [5,9]. When present however, the hormones typically found to be associated with these symptoms are epinephrine or norepinephrine.…”