New Biology of Pheochromocytoma and Paraganglioma

Pacák, Karel

doi:10.1016/j.eprac.2022.09.003

Cited by 14 publications

(7 citation statements)

References 131 publications

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“…3 Used to restore sinus rhythm. 4 In case of hypotension and suspected beta2-adrenoceptor overstimulation. NDHPCCB, Nondihydropyridine Calcium Channel Blockers; ST, Sinus Tachycardia; AF/AFL, Atrial Fibrillation/Atrial Flutter; VT, Ventricular Tachycardia; MCS, Mechanical Circulatory Support.…”

Section: Publisher's Notementioning

confidence: 99%

“…The effect of supraphysiological levels of epinephrine and norepinephrine on the heart and vessels highly depend on the secretory profile of the PPGL (adrenergic vs. noradrenergic phenotype, episodic via. continuous release) (4). The most common, yet alone nonspecific sign of PPGL: hypertension, among other factors, may lead to myocardial hypoxia with various clinical manifestations: acute (takotsubo, ischemic) and chronic (dilated, hypertrophic) cardiomyopathy (5)(6)(7)(8).…”

Section: Introductionmentioning

confidence: 99%

“…Catecholamine-induced cardiomyopathy in PPGL (CICMPP) is potentially fatal, but an uncommon complication, with a prevalence of 8–11% of patients with a PPGL ( 9 , 10 ). Although CICMPP is rarely the initial manifestation of PPGL, it is obligatory to rule out PPGL in patients with heart failure and paroxysmal symptoms: profuse diaphoresis, headaches, pallor, tremor, palpitations, and episodic hypertension, but also hyperglycemia in young patients with normal body mass index (BMI) ( 4 , 10 – 12 ).…”

Section: Introductionmentioning

confidence: 99%

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Pheochromocytoma/paraganglioma-associated cardiomyopathy

2023

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Pheochromocytoma/paraganglioma (PPGL) are neuroendocrine tumors that frequently produce and release catecholamines. Catecholamine excess can manifest in several cardiovascular syndromes, including cardiomyopathy. PPGL-induced cardiomyopathies occur in up to 11% of cases and are most often associated with an adrenal pheochromocytoma (90%) and rarely with a paraganglioma derived from the sympathetic ganglia (10%). PPGL-associated cardiomyopathies can be chronic or acute, with takotsubo cardiomyopathy being the most often reported. These two types of PPGL-induced cardiomyopathy seem to have different pathophysiological backgrounds. Acute catecholaminergic stress inundates myocardial β-adrenoceptors and leads to left ventricle stunning and slight histological apoptosis. In chronic cardiomyopathy, prolonged catecholamine exposure leads to extended myocardial fibrosis, inflammation, and necrosis, and ultimately it causes dilated cardiomyopathy with a low ejection fraction. Sometimes, especially in cases associated with hypertension, hypertrophic cardiomyopathy can develop. The prognosis appears to be worse in chronic cases with a higher hospital mortality rate, higher cardiogenic shock rate at initial presentation, and lower left ventricular recovery rate after surgery. Therefore, establishing the correct diagnosis at an early stage of a PPGL is essential. This mini-review summarizes current data on pathophysiological pathways of cardiac damage caused by catecholamines, the clinical presentation of PPGL-induced cardiomyopathies, and discusses treatment options.

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Section: Publisher's Notementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Pheochromocytoma/paraganglioma-associated cardiomyopathy

2023

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“…The aim of imaging is to determine whether the disease is resectable, unifocal, or multifocal and to identify metastatic recurrences and association with non-PPGL tumors ( 18 – 20 ). PHEOs typically exhibit an attenuation density on unenhanced CT >10 HU (often > 20 HU) ( 21 , 22 ). They frequently contain a central area of necrosis and may have a “ring sign” ( 23 ), which is peripheral contrast enhancement at the edge of the lesion (observed in 40% of cases compared to 2-3% for other tumors).…”

Section: Pheochromocytomasmentioning

confidence: 99%

Clinical utility of nuclear imaging in the evaluation of pediatric adrenal neoplasms

et al. 2023

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Adrenal neoplasms rarely occur in children. They can be diagnosed in the presence of endocrine, metabolic or neurological problems, an abdominal mass, more rarely an adrenal incidentaloma, or in the context of an adrenal mass discovered in the evaluation of childhood cancer including hematologic malignancy. According to standard medical practice, pediatric malignancies are almost always evaluated by 18F-fluorodeoxyglucose positron emission tomography with computed tomography ([18F]FDG PET/CT). Nuclear imaging using specific radiotracers is also an important tool for diagnosing and staging neuroblastoma, pheochromocytoma, hormone hypersecretion, or indeterminate adrenal masses. The Hippocratic oath “primum non nocere” encourages limitation of radiation in children per the ALARA concept (as low as reasonably achievable) but should not lead to the under-use of nuclear imaging because of the potential risk of inaccurate diagnosis or underestimation of the extent of disease. As in adults, nuclear imaging in children should be performed in conjunction with hormone evaluation and morphological imaging.

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“…PPGL sú nádory produkujúce katecholamíny -adrenalín a/alebo noradrenalín, v prípade extraadrenálnych formiem zriedkavo dopamín. Katecholamíny sú secernované do krvného obehu u cca 70 % pacientov, v ostatných prípadoch sa v nádore ukladajú a následne spätne degradujú (1,2).…”

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Catecholamine induced cardiomyopathies in patients with pheochromocytoma

Lazúrová

2024

Vnitr Lek

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Feochromocytóm/paraganglióm (PPGL) je zriedkavý neuroendokrinný nádor z chromafinných buniek sympatikových ganglií. V 90 % prípadov je lokalizovaný v dreni nadobličiek (feochromocytóm), ak je uložený extraadrenálne (cca 10 %), ide o paraganglióm. PPGL sú nádory produkujúce katecholamíny -adrenalín a/alebo noradrenalín, v prípade extraadrenálnych foriem zriedkavo dopamín. Nadprodukcia týchto hormónov sa u väčšiny pacientov prejaví kardiovaskulárnym ochorením, najčastejšie artériovou hypertenziou. U 8 -11 % pacientov s PPGL dochádza v dôsledku kardiotoxicity katecholamínov k poškodeniu srdca. Katecholamínmi indukované kardiomyopatie (CICMP) predstavujú závažné, až potenciálne fatálne komplikácie PPGL. Klinický obraz je variabilný, od asymptomatických pacientov až po dramatický priebeh s ťažkým srdcovým zlyhávaním s pľúcnym edémom alebo kardiogénnym šokom. Existujú tri formy CICMP asociovaných s PPGL. Akútna, takotsubo-like kardiomyopatia, typická pre adrenalín produkujúce tumory, a dva chronické typy -dilatačná kardiomyopatia a hypertrofická kardiomyopatia, typická pre nádory produkujúce noradrenalín. Včasná diagnostika a adekvátny manažment vedie u väčšiny pacientov k významnej regresii kardiálnych zmien. Kauzálna liečba spočíva v chirurgickom odstránení PPGL. Inoperabilné alebo metastatické formy PPGL majú častejšie recidívy CICMP a významne horšiu prognózu.

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New Biology of Pheochromocytoma and Paraganglioma

Cited by 14 publications

References 131 publications

Pheochromocytoma/paraganglioma-associated cardiomyopathy

Pheochromocytoma/paraganglioma-associated cardiomyopathy

Clinical utility of nuclear imaging in the evaluation of pediatric adrenal neoplasms

Catecholamine induced cardiomyopathies in patients with pheochromocytoma

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