Catecholamine Metabolism of a Ganglioneuroma: Correlation with Electronmicrographs

Abstract

ExtractStudies of the catecholamine metabolism in a patient having a functional ganglioneuroma are reported. Biochemical determinations of catecholamines and metabolites were correlated with histochemical and electron microscopic examination of the tumor. Increased urinary excretion of 3-methoxy-4-hydroxy mandelic acid, homovanillic acid, and dopamine was found prior to excision of the tumor. There was no significant increase in excretion of normetanephrine-metanephrine or of norepinephrine-epinephrine. Concentration of norepinephrine and epinephrine in the tumor tissue was 0.095 mglg. This concentration is greater than that found in neuroblastomas, but less than that found in pheochromocytomas. Normetanephrine-metanephrine was detected in the tumor tissue, indicating degradation of norepinephrine by catechol-0-methyl transferase. Computed turnover of norepinephrine in the tumor tissue was 6.85 hours.Catecholamines were present within heavy mitochondria1 fractions prepared from tumor tissue; the spontaneous release of norepinephrine into the suspending medium was determined during incubation under standard conditions. The data indicate that rapid turnover of norepinephrine was not related to accelerated spontaneous release from catecholamine granules. Electron microscopic examination revealed ganglion cells and a complex neuropil. Dense core vesicles were present in many of the neural processes and in ganglion cells. Synaptic junctions between neural processes and between neural processes and ganglion cells were seen. Nerve bundles containing neural processes were present. There was wide variation in the cross sectional diameter of the processes.The number of Type I dense core vesicles of this ganglioneuroma were decreased in comparison with those found in pheochromocytomas and increased in relation to neuroblastomas.The synapse-like areas of membrane specialization observed in this tumor may have been part of the mechanism by which differentiation of the ganglion cells was directed. The high rate of turnover of catecholamines does not seem to be dependent upon an increased rate of spontaneous release of catecholamines from isolated secretory vesicles.

Primary Retroperitoneal Ganglioneuroma: A Retrospective Cohort Study of 32 Patients

“…Among our cases, eight patients had abdominal pain and one patient had hand numbness and neck pain, which were in accordance with the tumor locations and sizes and can be explained by the mass effects. What is more, the tumors may occasionally secrete hormones including catecholamine, vasoactive intestinal peptide, and androgen, resulting in hypertension, sweating, diarrhea, or virilization (13,(19)(20)(21)(22)(23). These suggest that GNs can contain functional cells or mix with adenoma tissues, but the exact mechanism remains to be explored.…”

Total laparoscopic excision of retroperitoneal ganglioneuroma: A case report

“…In this case, gastrointestinal symptoms, such as nausea, vomiting, and anorexia were the patient's chief complaints. Although ganglioneuroma is endocrine-inactive, some ganglioneuromas produce symptoms such as diarrhea, hyperhidrosis, flushing, and hypertension due to the production of catecholamines and vasoactive intestinal peptides (VIP) [ 8 ]. On imaging, CT often shows homogenous low density mass, while contrast-enhanced CT shows poor contrast effects.…”

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

“…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].…”

“…In the cases of incomplete resection, the patient must be monitored closely for possible tumor progression, with particular concern if the residual tumor is 2 cm or greater as these are more likely to result in progression [5,7]. Additionally, for metabolically active ganglioneuromas, those that are completely excised show normalization of hormone levels after removal, but those that are not completely excised may be concerning for persistent elevation [9,[14], [15], [16], [17], [18], [19], [20], [21], [22], [23], [24], [25], [26], [27], [28], [29]]. Thus, follow up for these patients may include imaging studies, measurement of the levels of active compounds, and medical management.…”