Gender-related differences in the clinical presentation of malignant and benign pheochromocytoma

Lai, Edwin W.; Perera, Shiromi M.; Havekes, Bas; Timmers, Henri J L M; Brouwers, Frederieke M.; McElroy, Beverly; Adams, Karen T.; Wesley, Robert; Eisenhofer, Graeme; Pacák, Karel

doi:10.1007/s12020-008-9108-4

Cited by 19 publications

(12 citation statements)

References 25 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…In keeping with this concept, patients with epinephrine-producing adrenergic tumors were characterized by an overall higher symptom score and in particular more tremor, pallor and panic/anxiety than patients with PPGLs that did not produce epinephrine. Other findings consistent with those of previous reports include the higher ages of disease presentation for patients with adrenergic than other PPGLs (33), higher reported prevalence of signs and symptoms in females than males (34), and the expectedly higher prevalences of hypertension and biochemical indices of catecholamine excess in patients with PPGLs suspected due to signs and symptoms compared to other indications (11,23). .…”

Section: Discussionsupporting

confidence: 90%

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

Self Cite

View full text Add to dashboard Cite

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.

show abstract

Section: Discussionsupporting

confidence: 90%

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

Self Cite

View full text Add to dashboard Cite

show abstract

“…Based on our definition for hypertension, 4 articles were excluded because they were done before 1993, and the definition of hypertension was not specified, and there was no answer to our email for definition. All-but one-of the studies were based on medical records of the patients; the study of W. Lai et al [ 19 ] was based on questionnaire from the patients after diagnosis of pheochromocytoma, which could make recall bias; so, this study was excluded from data analysis. Finally, 37 articles were analyzed (Fig.…”

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

View full text Add to dashboard Cite

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...

show abstract

“…Some have indicated that patient sex may influence the chance of malignancy but this seems unlikely [16,24,25]. By contrast, persistent hypertension post-operatively increases the risk of malignancy (HR 5.3; P B 0.01) as it may represent occult metastases [11,23].…”

Section: Patient and Tumor Characteristicsmentioning

confidence: 99%

Pheochromocytomas and paragangliomas: assessment of malignant potential

Korevaar

Grossman

2011

Endocrine

View full text Add to dashboard Cite

Pheochromocytomas and paragangliomas (PPGLs) are rare catecholamine-secreting tumors which arise from the adrenal glands or sympathetic neuronal tissue. Malignant transformation of these tumors occurs in a significant proportion and may therefore lower overall survival rates. In patients with PPGLs it is impossible to identify malignant disease without the presence of metastatic disease, something which can occur as long as 20 years after initial surgery. Early identification of malignant disease would necessitate a more aggressive treatment approach, something which may result in better disease outcome. We have therefore reviewed possible predictors of malignancy and current developments in order to help clinicians to swiftly assess malignant potential in patients with PPGLs. Currently, there is no absolute marker which can objectively reflect malignant potential. Tumor size is the most reliable predictor and should therefore be used as the baseline characteristic. The combination of various clinical markers (extra-adrenal disease and post-operative hypertension), biochemical markers (high dopamine, high norepinephrine and epinephrine to total catecholamine ratio) and/or histological markers (SNAIL, microRNAs and/or microarray results) can raise or lower the suspicion of malignancy. Furthermore, we discuss how clinical markers may affect biochemical results linked to malignancy, how biochemical results may distinguish hereditary syndromes, the role of imaging in determining malignant potential and tumor detection, and recent results of proposed histological markers.

show abstract

Gender-related differences in the clinical presentation of malignant and benign pheochromocytoma

Cited by 19 publications

References 25 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

Pheochromocytomas and paragangliomas: assessment of malignant potential

Contact Info

Product

Resources

About