Pheochromocytomas detected by biochemical screening in predisposed subjects are associated with lower prevalence of clinical and biochemical manifestations and smaller tumors than pheochromocytomas detected by signs and symptoms

Duinen, Nicolette van; Steenvoorden, Daniëlle; Bonsing, Bert A.; Vuyk, Jaap; Vriends, Annette H. J. T.; Jansen, Jeroen C.; Romijn, Johannes A.; Corssmit, Eleonora P M

doi:10.1530/eje-10-0114

Cited by 18 publications

(8 citation statements)

References 28 publications

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“…Nevertheless, although present at higher prevalences in patients with than without PPGLs the triad also exhibited a relative high frequency among those without PPGLs illustrating its non-specific nature. Subsequent reports, although limited to patients with PPGLs, have indicated relatively lower proportions of patients presenting with the "classic triad" (21)(22)(23). This is in keeping with the findings here that the symptom triad was present in less than 25% of patients with PPGLs and at prevalences on average only 2-fold higher than in patients without PPGLs.…”

Section: Relationships Of Catecholamine Biochemistry With Clinical Fesupporting

confidence: 89%

Pheochromocytoma and paraganglioma: clinical feature-based disease probability in relation to catecholamine biochemistry and reason for disease suspicion

Geroula

Deutschbein

Langton

et al. 2019

European Journal of Endocrinology

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Objective Hypertension and symptoms of catecholamine excess are features of pheochromocytomas and paragangliomas (PPGLs). This prospective observational cohort study assessed whether differences in presenting features in patients tested for PPGLs might assist establishing likelihood of disease. Design and methods Patients were tested for PPGLs because of signs and symptoms, an incidental mass on imaging or routine surveillance due to previous history or hereditary risk. Patients with (n = 245) compared to without (n = 1820) PPGLs were identified on follow-up. Differences in presenting features were then examined to assess the probability of disease and relationships to catecholamine excess. Results Hyperhidrosis, palpitations, pallor, tremor and nausea were 30–90% more prevalent (P < 0.001) among patients with than without PPGLs, whereas headache, flushing and other symptoms showed little or no differences. Although heart rates were higher (P < 0.0001) in patients with than without PPGLs, blood pressures were not higher and were positively correlated to BMI, which was lower (P < 0.0001) in patients with than without PPGLs. From these differences in clinical features, a score system was established that indicated a 5.8-fold higher probability of PPGLs in patients with high than low scores. Higher scores among patients with PPGLs were associated, independently of tumor size, with higher biochemical indices of catecholamine excess. Conclusions This study identifies a complex of five signs and symptoms combined with lower BMI and elevated heart rate as key features in patients with PPGLs. Prevalences of these features, which reflect variable tumoral catecholamine production, may be used to triage patients according to likelihood of disease.

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Section: Relationships Of Catecholamine Biochemistry With Clinical Fesupporting

confidence: 89%

Pheochromocytoma and paraganglioma: clinical feature-based disease probability in relation to catecholamine biochemistry and reason for disease suspicion

Geroula

Deutschbein

Langton

et al. 2019

European Journal of Endocrinology

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“…1994–2003 17 (19 at first. 2 lost to follow up) 47 (17–72) 6,11 [ 35 ] Van Duinen et al 2010 Leiden university medical center, Netherland, 1975–2008 28 patients whom the pheochromocytoma was diagnosed based on signs and symptoms 47 – [ 36 ] Bernard Goldny et al, 2001 Department of General Surgery of the University of Munster Hospital, Germany. 1967–1998 133 41.4 (2–75) 68,65 [ 37 ] I.M.…”

Section: Resultsmentioning

confidence: 99%

Does this patient have Pheochromocytoma? a systematic review of clinical signs and symptoms

Soltani

Pourian

Davani

2016

J Diabetes Metab Disord

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ContextPheochromocytoma is a rare disease but with high mortality if it is not being diagnosed early. Several biochemical tests with high accuracy have been obtained, but the clinical threshold for request of these tests is not determined clearly.ObjectivesTo determine the Likelihood Ratios of clinical symptoms and signs in diagnosing pheochromocytoma. And also meta-analysis of their sensitivity in this disease.Data sourcesMEDLINE was searched for relevant English-language articles dated 1960 to February 2014. Bibliographies were searched to find additional articles.Study selectionWe included original studies describing the sensitivity and/or likelihood ratios of signs and symptoms in clinical suspicion of pheochromocytoma. Their method of diagnosis should have been based on pathology. We excluded specific subtypes or syndromes related to pheochromocytoma, or specific ages or gender. Also we excluded studies before 1993 (JNC5) which no definition of hypertension was presented. 37 articles were chosen finally.Data extractionTwo authors reviewed data from articles independently and gave discrepancies to third author for decision. The aim was extraction of raw numbers of patients having defined signs or symptoms, and draw 2 × 2 tables if data available. We meta-analyzed sensitivities by Statsdirect and Likelihood Ratios by Meta-disc soft wares. Because our data was heterogeneous based on I2 > 50 % (except negative Likelihood ratio of hypertension), we used random effect model for doing meta-analysis. We checked publication bias by drawing Funnel plot for each sign/symptom, and also Egger test.Data synthesisThe most prevalent signs and symptoms reported were hypertension (pooled sensitivity of 80.7 %), headache (pooled sensitivity of 60.4 %), palpitation (pooled sensitivity of 59.3 %) and diaphoresis (pooled sensitivity of 52.4 %). The definition of orthostatic hypotension was different among studies. The sensitivity was 23–50 %.Paroxysmal hypertension, chest pain, flushing, and weakness were the signs/symptoms which had publication bias based on Funnel plot and Egger test (P value < 0.05). Seven of the articles had control group, and could be used for calculating LR of signs/symptoms. Diaphoresis (LR+ 2.2, LR-0.45), Palpitation (LR+ 1.9, LR-0.52) and headache (LR+ 1.6, LR-0.24) were significant symptoms in clinical diagnosis of pheochromocytoma. Other signs and symptoms had been reported in only one study and could not have been meta-analyzed. Classic triad of headache, palpitation and diaphoresis in hypertensive patients had the LR+ 6.312 (95 % CI 0.217–183.217) and LR-0.139 (95 % CI 0.059–0.331). Surprisingly, hypertension was not important in clinical suspicion of pheochromocytoma, and even normotension increased the probability of the disease.ConclusionsBy available data, there is no single clinical finding that has significant value in diagnosis or excluding pheochromocytoma. Combination of certain symptoms, signs and para-clinical exams is more valuable for physicians. Further studies should be done, to specif...

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“…1994–2003 17 (19 at first. 2 lost to follow up) 47 (17–72) 6,11 [ 35 ] Van Duinen et al 2010 Leiden university medical center, Netherland, 1975–2008 28 patients whom the pheochromocytoma was diagnosed based on signs and symptoms 47 --- [ 36 ] Bernard Goldny et al, 2001 Department of General Surgery of the University of Munster Hospital, Germany. 1967–1998 133 41.4 (2–75) 68,65 [ 37 ] I.M.…”

Section: Resultsmentioning

confidence: 99%

Does this patient have pheochromocytoma? A systematic review of clinical signs and symptoms

Pourian

Mostafazadeh

Soltani

2015

J Diabetes Metab Disord

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ContextPheochromocytoma is a rare disease but with high mortality if it is not being diagnosed early. Several biochemical tests with high accuracy have been obtained, but the clinical threshold for request of these tests is not determined clearly.ObjectivesTo determine the Likelihood Ratios of clinical symptoms and signs in diagnosing pheochromocytoma. And also meta-analysis of their sensitivity in this disease.Data sourcesMEDLINE was searched for relevant English-language articles dated 1960 to February 2014. Bibliographies were searched to find additional articles.Study selectionWe included original studies describing the sensitivity and/or likelihood ratios of signs and symptoms in clinical suspicion of pheochromocytoma. Their method of diagnosis should have been based on pathology. We excluded specific subtypes or syndromes related to pheochromocytoma, or specific ages or gender. Also we excluded studies before 1993 (JNC5) which no definition of hypertension was presented. 37 articles were chosen finally.Data extractionTwo authors reviewed data from articles independently and gave discrepancies to third author for decision. The aim was extraction of raw numbers of patients having defined signs or symptoms, and draw 2 × 2 tables if data available. We meta-analyzed sensitivities by Statsdirect and Likelihood Ratios by Meta-disc soft wares. Because our data was heterogeneous based on I2 > 50 % (except negative Likelihood ratio of hypertension), we used random effect model for doing meta-analysis. We checked publication bias by drawing Funnel plot for each sign/symptom, and also Egger test.Data synthesisThe most prevalent signs and symptoms reported were hypertension (pooled sensitivity of 80.7 %), headache (pooled sensitivity of 60.4 %), palpitation (pooled sensitivity of 59.3 %) and diaphoresis (pooled sensitivity of 52.4 %). The definition of orthostatic hypotension was different among studies. The sensitivity was 23–50 %.Paroxysmal hypertension, chest pain, flushing, and weakness were the signs/symptoms which had publication bias based on Funnel plot and Egger test (P value < 0.05). Seven of the articles had control group, and could be used for calculating LR of signs/symptoms. Diaphoresis (LR+ 2.2, LR- 0.45), Palpitation (LR+ 1.9, LR- 0.52) and headache (LR+ 1.6, LR- 0.24) were significant symptoms in clinical diagnosis of pheochromocytoma. Other signs and symptoms had been reported in only one study and could not have been meta-analyzed. Classic triad of headache, palpitation and diaphoresis in hypertensive patients had the LR+ 6.312 (95 % CI 0.217–183.217) and LR- 0.139 (95 % CI 0.059–0.331). Surprisingly, hypertension was not important in clinical suspicion of pheochromocytoma, and even normotension increased the probability of the disease.ConclusionsBy available data, there is no single clinical finding that has significant value in diagnosis or excluding pheochromocytoma. Combination of certain symptoms, signs and para-clinical exams is more valuable for physicians. Further studies should be done, to sp...

show abstract

Pheochromocytomas detected by biochemical screening in predisposed subjects are associated with lower prevalence of clinical and biochemical manifestations and smaller tumors than pheochromocytomas detected by signs and symptoms

Cited by 18 publications

References 28 publications

Pheochromocytoma and paraganglioma: clinical feature-based disease probability in relation to catecholamine biochemistry and reason for disease suspicion

Pheochromocytoma and paraganglioma: clinical feature-based disease probability in relation to catecholamine biochemistry and reason for disease suspicion

Does this patient have Pheochromocytoma? a systematic review of clinical signs and symptoms

Does this patient have pheochromocytoma? A systematic review of clinical signs and symptoms

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