Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery

Gruber, Lucinda; Hartman, Robert P.; Thompson, Geoffrey B.; McKenzie, Travis J.; Lyden, Melanie L.; Dy, Benzon M.; Young, William F.; Bancos, Irina

doi:10.1210/jc.2018-01707

Cited by 134 publications

(99 citation statements)

References 11 publications

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“…Whereas previously affected patients were mainly diagnosed based on signs and symptoms, today the tumors are increasingly found incidentally as part of imaging studies for purposes unrelated to suspicion of PPGL (6,11,12). Due to recognition of an increasing number of pathogenic mutations and a high rate of disease recurrence, PPGLs are now being found during surveillance screening in patients with a genetic predisposition or past history of disease (11). Such patients can be asymptomatic and normotensive (13).…”

Section: Introductionmentioning

confidence: 99%

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: Introductionmentioning

confidence: 99%

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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“…PPGLs are more frequent in certain groups, for example, in patients with adrenal incidentalomas with 0.6% to 4.2% being affected but is otherwise generally rare . Most PPGLs are nowadays diagnosed due to an incidentaloma, then due to catecholamine excess symptoms and finally because of screening in a previously known familial syndrome (eg, multiple endocrine neoplasia type 2, von Hippel Lindau syndrome, neurofibromatosis type 1, and mutations in succinate dehydrogenase B, C, and D) Cushing's syndrome due to ectopic ACTH‐production from a PPGL can occasionally occur, and thus all adrenal tumors should have a 1 mg overnight dexamethasone suppression test to exclude cortisol excess . Sometimes an adrenal medullary hyperplasia may be the culprit of catecholamine excess …”

Section: Introductionmentioning

confidence: 99%

Clinical features, complications, and outcomes of exogenous and endogenous catecholamine‐triggered Takotsubo syndrome: A systematic review and meta‐analysis of 156 published cases

Y‐Hassan

Falhammar

2020

Clinical Cardiology

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Innumerable physical stress factors including externally administered catecholamines, and pheochromocytomas and paragangliomas (PPGLs) have been reported to trigger Takotsubo syndrome (TS). A systematic search of PubMed/MEDLINE identified 156 patients with catecholamine-induced TS up to December 2017. Data were compared within the catecholamine-induced TS cohort, but some comparisons were also done to a previously published large all-TS cohort (n = 1750). The mean age was 46.4 ± 16.4 years (72.3% women). The clinical presentation was dramatic with high complication rates in (68.2%, n = 103; multiple complications 34.6%, n = 54). The most common TS ballooning pattern was apical or mid-apical (45.2%, n = 69), followed by basal pattern (28.8%, n = 45), global pattern (16.0%, n = 25), midventricular (8.3%, n = 13), focal (0.6%, n = 1), and unidentified pattern (1.9%, n = 3).There was an increase in the prevalence of apical sparing ballooning pattern compared to all-TS population (37.7% vs 18.3%, P < .00001). Higher complication rates were observed in TS with global ballooning pattern compared to apical ballooning pattern (23/25, 92% vs 38/65, 58.5%; P = .0022). Higher complication rates were observed in patients with age < 50 years than patients >50 years (73/92, 79.3% vs 29/56, 51.8%, P = 0.0009). Recurrence occurred exclusively in patients with PPGLinduced TS (18/107 patients, 16.8%). PPGL-induced TS was characterized by more global ballooning's pattern (22/104, 21.2% vs 3/49, 6.1%, P = 0.02), and lower left ventricular ejection fraction (25.54 ± 11.3 vs 31.82 ± 9.93, P = 0.0072) compared to exogenous catecholamine-induced TS. In conclusion, catecholamine-induced TS was characterized by a dramatic clinical presentation with extensive left ventricular dysfunction, and high complication rate. K E Y W O R D S broken heart syndrome, epinephrine, myocardial stunning, norepinephrine, paraganglioma, pheochromocytoma, Takotsubo

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“…Pheochromocytomas, although rare, are becoming more common due to incidental detection on CT. Gruber et al identified that incidental pheochromocytomas were typically found in older patients, with fewer symptoms and with a smaller degree in elevation of urine and serum metanephrines compared to those diagnosed based on symptoms. Incidentally discovered pheochromocytomas were also found to require less cumulative phenoxybenzamine compared to those with symptoms [2].…”

Section: Discussionmentioning

confidence: 94%

Adrenal Adenoma Anarchy: A Case of an ACTH-Secreting Pheochromocytoma

Lee

Wan

Chormanski

et al. 2020

Case Reports in Endocrinology

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Introduction. Pheochromocytomas are rare neuroendocrine tumors that arise from sympathetic adrenomedullary chromaffin tissue. Depending on the amount of catecholamines they secrete, they have variable presentations. There have been reported cases of adrenocorticotrophic (ACTH) secreting pheochromocytomas that present with severe Cushing syndrome. Here, we present a pheochromocytoma with adrenocorticotrophic hormone (ACTH) cosecretion, which due to its rarity and variable presentation, may be a diagnostic challenge. Presentation. A 64-year-old woman with history of colon cancer presented with new-onset diabetes, worsening hot flashes, and hypertension. On CT imaging she had an enlarging right adrenal nodule (1.7 cm) with 60 Hounsfield units of attenuation and no PET avidity. Biochemical evaluation showed elevated urinary and plasma metanephrines, elevated plasma cortisol levels despite dexamethasone suppression, elevated late-night salivary cortisol, and high-normal adrenocorticotrophic hormone. The patient underwent laproscopic right adrenalectomy, and pathology confirmed pheochromocytoma. Her lab abnormalities and symptoms of hot flashes and hypertension improved postoperatively. Conclusion. This case demonstrates an unusual ACTH-secreting pheochromocytoma with subtle presentation and highlights the importance of obtaining a complete biochemical evaluation of incidental adrenal adenomas.

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Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery

Cited by 134 publications

References 11 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

Clinical features, complications, and outcomes of exogenous and endogenous catecholamine‐triggered Takotsubo syndrome: A systematic review and meta‐analysis of 156 published cases

Adrenal Adenoma Anarchy: A Case of an ACTH-Secreting Pheochromocytoma

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