Phaeochromocytoma with Normal Urinary Catecholamines: The Potential Value of Urinary Free Metadrenalines

Davidson, D F

doi:10.1177/000456320203900603

Cited by 37 publications

(20 citation statements)

References 38 publications

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“…This secretion is independent of highly variable catecholamine release caused by the tumour or by sympathoadrenal excitation (35). Comparisons of different biochemical tests established that both plasma free metanephrines or urinary fractionated metanephrines offer a higher sensitivity for the diagnosis of PPGL than other traditional tests (24,25,33,35,36,37,38,39,40,41). Plasma free metanephrines offers a sensitivity up to 96-99% (Table 2).…”

Section: Which Test Is the Best?mentioning

confidence: 98%

DIAGNOSIS OF ENDOCRINE DISEASE: Biochemical diagnosis of phaeochromocytoma and paraganglioma

Berkel

Lenders

Timmers

2014

European Journal of Endocrinology

144

112

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Adrenal phaechromocytomas and extra-adrenal sympathetic paragangliomas (PPGLs) are rare neuroendocrine tumours, characterised by production of the catecholamines: noradrenaline, adrenaline and dopamine. Tumoural secretion of catecholamines determines their clinical presentation which is highly variable among patients. Up to 10-15% of patients present entirely asymptomatic and in 5% of all adrenal incidentalomas a PPGL is found. Therefore, prompt diagnosis of PPGL remains a challenge for every clinician. Early consideration of the presence of a PPGL is of utmost importance, because missing the diagnosis can be devastating due to potential lethal cardiovascular complications of disease. First step in diagnosis is proper biochemical analysis to confirm or refute the presence of excess production of catecholamines or their metabolites. Biochemical testing is not only indicated in symptomatic patients but also in asymptomatic patients with adrenal incidentalomas or identified genetic predispositions. Measurements of metanephrines in plasma or urine offer the best diagnostic performance and are the tests of first choice. Paying attention to sampling conditions, patient preparation and use of interfering medications is important, as these factors can largely influence test results. When initial test results are inconclusive, additional tests can be performed, such as the clonidine suppression test. Test results can also be used for estimation of tumour size or prediction of tumour location and underlying genotype. Furthermore, tumoural production of 3-methoxytyramine is associated with presence of an underlying SDHB mutation and may be a biomarker of malignancy.

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Section: Which Test Is the Best?mentioning

confidence: 98%

DIAGNOSIS OF ENDOCRINE DISEASE: Biochemical diagnosis of phaeochromocytoma and paraganglioma

Berkel

Lenders

Timmers

2014

European Journal of Endocrinology

144

112

View full text Add to dashboard Cite

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“…23 Such data may be normalized, for example by logarithmic transformation or by employing non-parametric statistics. 15,16,22,23 The upper reference limits for urinary catecholamines and metabolites used in the present study were established taking account of both factors. In addition, we determined their practical validity by a peer-reviewed study using receiver operating characteristic curve analysis, 11 and our URL values are similar or identical to those quoted by several other laboratories employing HPLC techniques.…”

Section: Discussionmentioning

confidence: 99%

“…15 The reference intervals for adults were NA (,900 nmol/24 h), AD (,230 nmol/24 h), DA (,3300 nmol/24 h), 16 VMA (,35 mmol/24 h), HVA (,40 mmol/24 h), 17 fNMA (,650 nmol/24 h) and fMA (,350 nmol/24 h). 15 In the case of random urines from children, results were expressed as millimole per mole creatinine, and the various age-related upper reference limits were as previously described. 14 Statistical analysis was performed using the MedCalc statistical software package (version 11.3.3.; MedCalc Software, Mariakerke, Belgium).…”

Section: Methodsmentioning

confidence: 99%

Urinary free (unconjugated) metadrenalines in different hereditary forms of catecholamine-secreting phaeochromocytoma/paraganglioma

Davidson

Bradshaw²,

Perry

et al. 2012

Ann Clin Biochem

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Background: Catecholamine-producing neuroendocrine tumours are found in chromaffin cells of the adrenal medulla ( phaeochromocytoma) or extra-adrenal paraganglia ( paraganglioma), known collectively as PPGLs. In approximately a quarter or more of cases of PPGL, these rare tumours arise as a result of germline mutations of several tumour susceptibility genes. At the Crosshouse laboratory, urine tests include free metadrenalines (fMAs) (also known as free metanephrines) which demonstrate superior sensitivity over that obtained by urinary vanillyl mandelic acid, catecholamines or plasma catecholamines in the diagnosis of PPGL. This retrospective audit was to determine if urinary fMAs offered discrimination among the hereditary forms of PPGL. Conclusions:The ratio of the multiples of URL for fMA/fNMA displayed a clearer separation of MEN 2 patients from those with SDHB/D or VHL than did the equivalent AD/NA ratio.

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“…With improved analytical sensitivity of modern LC-ECD and LC-MS/MS instruments, it has become possible to measure urine free metanephrines (16,24 ), which also provide improved diagnosis of PPGLs compared to catecholamines and VMA (25 ). Such measurements not only avoid the quality assurance problems associated with measurements of deconjugated metanephrines, but also offer theoretical advantages related to measurements of free metabolites formed within chromaffin cells and their tumor derivatives (18,23 ).…”

Section: Free Vs Conjugated Metanephrinesmentioning

confidence: 99%

Laboratory Evaluation of Pheochromocytoma and Paraganglioma

Eisenhofer

Peitzsch²

2014

Clinical Chemistry

160

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BACKGROUND: Pheochromocytomas and paragangliomas (PPGLs) are potentially lethal yet usually surgically curable causes of endocrine hypertension; therefore, once clinical suspicion is aroused it is imperative that clinicians choose the most appropriate laboratory tests to identify the tumors. CONTENT:Compelling evidence now indicates that initial screening for PPGLs should include measurements of plasma free metanephrines or urine fractionated metanephrines. LC-MS/MS offers numerous advantages over other analytical methods and is the method of choice when measurements include methoxytyramine, the O-methylated metabolite of dopamine. The plasma test offers advantages over the urine test, although it is rarely implemented correctly, rendering the urine test preferable for mainstream use. To ensure optimum diagnostic sensitivity for the plasma test, reference intervals must be established for blood samples collected after 30 min of supine rest and after an overnight fast when measurements include methoxytyramine. Similarly collected blood samples during screening, together with use of age-adjusted reference intervals, further minimize false-positive results. Extents and patterns of increases in plasma normetanephrine, metanephrine, and methoxytyramine can additionally help predict size and adrenal vs extraadrenal locations of tumors, as well as presence of metastases and underlying germline mutations of tumor susceptibility genes.

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Phaeochromocytoma with Normal Urinary Catecholamines: The Potential Value of Urinary Free Metadrenalines

Cited by 37 publications

References 38 publications

DIAGNOSIS OF ENDOCRINE DISEASE: Biochemical diagnosis of phaeochromocytoma and paraganglioma

DIAGNOSIS OF ENDOCRINE DISEASE: Biochemical diagnosis of phaeochromocytoma and paraganglioma

Urinary free (unconjugated) metadrenalines in different hereditary forms of catecholamine-secreting phaeochromocytoma/paraganglioma

Laboratory Evaluation of Pheochromocytoma and Paraganglioma

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