Spectrum of Catechol Amine Biochemistry in Patients with Neuroblastoma

Studnitz, W. von; Käser, H; Sjoerdsma, Albert

doi:10.1056/nejm196308012690503

Cited by 92 publications

(16 citation statements)

References 15 publications

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“…The cystathionine nor elevated catecholamine degradation most thoroughly described of these are the catecholaproducts in his urine. The tumor, recovered surgically mine degradation products [19]. Cystathionine may from his abdomen, contained high levels of a comalso be found in the urine [6,7] as well as in the pound identified as S-adenosylmethionine.…”

Section: Introductionmentioning

confidence: 99%

S-Adenosylmethionine in a Neuroblastoma

Lyon

1971

Pediatr Res

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Section: Introductionmentioning

confidence: 99%

S-Adenosylmethionine in a Neuroblastoma

Lyon

1971

Pediatr Res

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“…Increased excretion of catecholamines and related metabolites in urine has been found in a number of patients with ganglioneuroma [16,19,23,27,28,29,30,41,47,54,56] ; however, such an increase has not been found in all cases of ganglioneuroma. KASER [27] reported normal urine VMA excretion in several patients with ganglioneuroma; however, values for urinary excretion of NE, dopamine, DOPA, HVA, and of NMN and MN were not reported.…”

Section: Discussionmentioning

confidence: 96%

“…Increased excretion of HVA in several cases indicated that dopamine and DOPA, intermediates in the synthesis of NE, were released from the tumor and metabolized elsewhere or, alternatively, were degraded within the tumor and then released. In this respect, ganglioneuromas are more similar to neuroblastomas than to pheochromocytomas [54]. Increased excretion of NMN and MN is found uniformly in patients with pheochromocytoma, but may not be found in patients with neuroblastoma or ganglioneuroma, despite a marked increase in VMA excretion [43].…”

Section: Discussionmentioning

confidence: 99%

Catecholamine Metabolism of a Ganglioneuroma: Correlation with Electronmicrographs

et al. 1969

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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.

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“…Noradrenaline-secreting neuroblastoma produce hypertension in a minority of cases. 43 To explain this, Sadler and Ruthven 8,44 proposed that noradrenaline is metabolically degraded in tumor tissue or peripherally and only tiny quantities of free noradrenaline are liberated into the circulation. It would however seem prudent that when biopsy or surgical resection is to be performed in patients with noradrenaline producing tumors, pre-procedure α-adrenoceptor blockade is used as a means to avoid hypertensive crisis.…”

Section: Discussionmentioning

confidence: 99%

Functional Retroperitoneal Neuroblastoma and Ganglioneuroblastoma in Adults

Mahkamova¹,

Harrison²

2014

World Journal of Endocrine Surgery

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Ganglioneuroblastoma and neuroblastoma are malignant catecholamine-secreting tumors arising from neural crest tissue. Thirty-eight cases of functional retroperitoneal extra-adrenal neuroblastoma and ganglioneuroblastoma in adults are reported in English literature. These tumors behave more aggressively in adults compared to children and have very poor prognosis. We report four adults who presented with retroperitoneal extraadrenal ganglioneuroblastoma and neuroblastoma between 2008 and 2012. Median age at presentation was 28.5 years (21-40 years). One patient was in late stages of pregnancy. Three patients presented with local pressure effects from the tumor and one patients manifested clinical features of catecholamine excess. Biochemical analysis showed raised urinary dopamine excretion in all our patients. Urinary noradrenaline and meta nephrines were elevated in three cases. Computed tomography/magnetic resonance imaging/meta-iodobenzylguanidine (CT/MRI/MIBG) confirmed stage IIB disease in one patient, stage III disease one patient and stage IV in two cases. Preoperative biopsy confirmed diagnosis in two cases. Complete surgical resection was performed in patient with stage II B disease and partial surgical resection was feasible in other cases. Histological diagnosis was ganglioneuroblastoma in two cases and neuroblastoma in two cases. Meta-iodobenzylglunidine/radiotherapy and chemotherapy were used for residual disease. Urinary catecholamines and metanephrines normalized after complete tumor resection but remained elevated in residual disease. Survival for neuroblastoma was 15 and 21 months after the surgery and 29 months for ganglioneuroblastoma. One patient remains free of disease at 6 months. Functioning ganglioneuroblastoma and neuroblastoma are rare in adults and only one other case ganglioneuroblastoma in pregnancy is reported in the world literature. Measurement of plasma/urinary excretion of catecholamines and their precursors and metabolites is helpful for diagnosis of neuroblastoma and ganglioneuroblastoma. Due to late presentation surgical resection is unlikely to be curative. Three of our patients developed local recurrence of disease. Urinary/plasma assay are used for monitoring disease relapse. Long-term survival is unusual.

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Spectrum of Catechol Amine Biochemistry in Patients with Neuroblastoma

Cited by 92 publications

References 15 publications

S-Adenosylmethionine in a Neuroblastoma

S-Adenosylmethionine in a Neuroblastoma

Catecholamine Metabolism of a Ganglioneuroma: Correlation with Electronmicrographs

Functional Retroperitoneal Neuroblastoma and Ganglioneuroblastoma in Adults

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