The Relationship between Enzyme Activity and the Catecholamine Content and Secretion of Pheochromocytomas*

Feldman, Jerome M.; Blalock, J; Zern, Ruthann T.; Wells, Samuel A.

doi:10.1210/jcem-49-3-445

Cited by 45 publications

(16 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…This further supports the observation that paroxysmally secreting phaeochromocytomas are characterized by a high catecholamine content [i4, 22]. In contrast to Ziegler et al [37] and Feldman et al [9] serotonin could not be detected in this tumor.…”

Section: Discussionsupporting

confidence: 90%

A paroxysmally secreting phaeochromocytoma: Biochemical and clinical aspects

et al. 1983

View full text Add to dashboard Cite

Summary.One of the problems encountered with the diagnosis of a paroxysmally secreting phaeochromocytoma is the failure of the conventional chemical screening tests to demonstrate an increased urinary excretion of catecholamines and/or their metabolites. The usefulness of the estimation of plasma catecholamines in the diagnosis of this kind of phaeochromocytoma is demonstrated in a 64 years old male patient. In addition the mode of secretion of catecholamines from this phaeochromocytoma was investigated.The patient suffered from paroxysms characterized by the feeling of pressure in the throat, pain in the substernal area, palpitations, hypertension, flush and headache. Because of a normal excretion of catecholamines and vanillylmandelic acid in the 24-h collection of urine, estimated on several occasions, a phaeochromocytoma was excluded for about 6 years. The existence of a phaeochromocytoma could be verified by the measurement of plasma catecholamines. The plasma levels of noradrenaline and adrenaline in symptom-free intervals several days previous to the removal of the phaeochromocytoma were comparable to the levels found postoperatively up to 2.5 months. However, during 3 paroxysms highly elevated plasma levels of noradrenaline (up to 21.3 ng/ml) with only a moderate increase of adrenaline (up to 0.54 ng/ml) were measured. After removal of the predominantly noradrenatine producing tumor, which was localized in the right adrenal gland, the paroxysms disappeared.Dopamine/?-hydroxylase activity in plasma as a marker of an exocytotic release mechanism neither increased during a paroxysm nor decreased * This work was supported by the Jubil/iumsfond of the ~ster-reichische NationalbankOffprint requests to: Dr. Heide H6rtnagl (address see page 607) postoperatively. Biochemically the paroxysmally secreting phaeochromocytoma was characterized by a high storage capacity for catecholamines, by a proportionate release from noradrenaline and adrenaline producing cells and by a release mechanism which is based on diffusion of the catecholamine out of the cell rather than on exocytosis.

show abstract

Section: Discussionsupporting

confidence: 90%

A paroxysmally secreting phaeochromocytoma: Biochemical and clinical aspects

et al. 1983

View full text Add to dashboard Cite

show abstract

“…Interestingly, 6.1% of our phaeochromocytoma patients were normotensive, emphasising that the absence of hypertension does not rule out the presence of a cataecholamine-producing tumour that might result in life-threatening situations (15). One explanation may be that tumour-dependent hormone production may remain silent for a long period of time, because active cataecholamines can be converted into biologically inactive metanephrines by cataechol-Omethyltransferase within the tumour (8,16,17). Owing to the rare occurrence, the often unspecific signs and symptoms, and the low sensitivity of any symptoms and the diagnosis of phaeochromocytoma may be delayed.…”

Section: Discussionmentioning

confidence: 86%

Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma

Kopetschke¹,

Slisko²,

Kilisli³

et al. 2009

European Journal of Endocrinology

180

114

View full text Add to dashboard Cite

Context: Adrenal and extra-adrenal phaeochromocytoma are chromaffin cell-derived tumours that are discovered due to classical symptom triad with headache, sweating and palpitations combined with persistent or paroxysmal hypertension. However, an increasing proportion of phaeochromocytoma seems to be discovered incidentally upon abdominal imaging. Objective: To specify the exact circumstances of discovery of adrenal and extra-adrenal phaeochromocytoma. Design and patients: Four German endocrine centres participated in this retrospective study. Medical records of 201 patients with adrenal and extra-adrenal phaeochromocytoma who were diagnosed between 1973 and 2007 were analyzed. Results: The typical triad of symptoms was found only in 10% of cases. Ten percent of patients presented were without clinical symptoms and 6.1% were normotensive. Documented blood pressure peaks occurred in 44.1% of cases. In 24 patients (12.2%), phaeochromocytoma was malignant. Before 1985, !10% of cases were incidentally discovered, whereas thereafter the frequency was O25% (29.4% of the total study population). Patients with incidentally detected phaeochromocytoma were significantly older (53.1G1.9 vs 47.0G1.3 years; P!0.05) and often had less blood pressure peaks (37.0 vs 70.7%; P!0.001) than patients in whom the diagnosis was suspected on clinical grounds. Of phaeochromocytomas 94.4% were intra-adrenal tumours, of which 12.9% were bilateral. Bilateral tumours were significantly smaller than unilateral tumours (36.6G14.7 vs 52.5G34.3 mm; P!0.05), whereas extra-adrenal tumours had a mean diameter of 52.6G28.7 mm. Conclusions: Owing to better availability and accessibility of imaging procedures, the number of incidentally discovered phaeochromocytoma is increasing and reaches nearly 30% in our study population. Every adrenal incidentaloma should be investigated for the presence of phaeochromocytoma.

show abstract

“…1,28 Helman et al 29 reported that neuropeptide Y mRNA is less frequently expressed in malignant than in benign pheochromocytomas, whereas de Senanayake et al 30 found higher plasma neuropeptide Y concentrations in adrenal than in extra-adrenal pheochromocytomas. The role of plasma neuropeptide Y in distinguishing benign from malignant pheochromocytoma has not yet been explored.…”

Section: Benign Versus Malignant Pheochromocytomamentioning

confidence: 99%

Malignant Pheochromocytoma

2000

View full text Add to dashboard Cite

Abstract-Chromaffin granule transmitters such as chromogranin A and catecholamines have been used in the diagnosis of pheochromocytoma, but the diagnostic and prognostic value of chromogranin A have not been explored in malignant pheochromocytoma. We evaluated these transmitters in patients with pheochromocytoma (nϭ27), both benign (nϭ13) and malignant (nϭ14). Patients with benign pheochromocytoma were studied before and after surgical excision (nϭ6), whereas patients with malignant pheochromocytoma were evaluated before and after combination chemotherapy with regular cycles of cyclophosphamide/dacarbazine/vincristine (nonrandomized trial in nϭ9). During treatment, patient responses to chemotherapy were divided according to anatomic and clinical criteria: responders (nϭ5) versus nonresponders (nϭ4). Plasma chromogranin A rose progressively (PϽ0.0001) from control subjects (48.0Ϯ3.0 ng/mL) to benign pheochromocytoma (188Ϯ40.5 ng/mL) to malignant pheochromocytoma (2932Ϯ960 ng/mL). Parallel changes were seen for plasma norepinephrine (PϽ0.0001), though plasma epinephrine was actually lower in malignant than benign pheochromocytoma (Pϭ0.0182). In bivariate analyses, chromogranin A, norepinephrine, and epinephrine discriminated between pheochromocytoma and control subjects (all PϽ0.0001), whereas in a multivariate analyses, norepinephrine was the best discriminator (Pϭ0.011). Chromogranin A was significantly different in benign versus malignant pheochromocytoma on both bivariate (Pϭ0.0003) and multivariate (Pϭ0.011) analyses. After excision of benign pheochromocytoma, chromogranin A (Pϭ0.028), norepinephrine (Pϭ0.047), and epinephrine (Pϭ0.037) all fell to values near normal. During chemotherapy of malignant pheochromocytoma (nϭ9), plasma chromogranin A (Pϭ0.047) and norepinephrine (Pϭ0.02) fell but not epinephrine. In 5 responders to chemotherapy, there were significant declines in chromogranin A (Pϭ0.03) and norepinephrine (Pϭ0.03) but not epinephrine; in 4 nonresponders, none of the transmitters changed. Plasma chromogranin A varied longitudinally with tumor response and relapse. We conclude that plasma chromogranin A is an effective tool in the diagnosis of pheochromocytoma, and markedly elevated chromogranin A may point to malignant pheochromocytoma. During chemotherapy of malignant pheochromocytoma, chromogranin A can be used to gauge tumor response and relapse. (Hypertension. 2000;36:1045-1052.)Key Words: plasma Ⅲ chromogranins Ⅲ norepinephrine Ⅲ epinephrine M alignant pheochromocytoma presents several difficulties in management, both in diagnosis 1,2 and in effective treatment. [2][3][4] Even after excision, the diagnosis of malignant pheochromocytoma cannot be made reliably on histological grounds 2 ; thus, extensive evaluations of tumor location are required to ascertain the presence of local or distant invasion or metastases by either surgical exploration, imaging, or regional venous blood sampling. 2 Specific biochemical diagnosis of malignant pheochromocytoma has received only limited attention. 1 Once d...

show abstract

The Relationship between Enzyme Activity and the Catecholamine Content and Secretion of Pheochromocytomas*

Cited by 45 publications

References 25 publications

A paroxysmally secreting phaeochromocytoma: Biochemical and clinical aspects

A paroxysmally secreting phaeochromocytoma: Biochemical and clinical aspects

Frequent incidental discovery of phaeochromocytoma: data from a German cohort of 201 phaeochromocytoma

Malignant Pheochromocytoma

Contact Info

Product

Resources

About