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. 2021 Mar 19;12(1):9.
doi: 10.1007/s12672-021-00404-x.

Metastatic pheochromocytoma and paraganglioma: signs and symptoms related to catecholamine secretion

Minghao Li  1 , Christina Pamporaki  1 , Stephanie M J Fliedner  2 , Henri J L M Timmers  3 , Svenja Nölting  4   5 , Felix Beuschlein  4   5 , Aleksander Prejbisz  6 , Hanna Remde  7 , Mercedes Robledo  8 , Stefan R Bornstein  1 , Jacques W M Lenders  1   3 , Graeme Eisenhofer  1   9 , Nicole Bechmann  10   11
Affiliations

Metastatic pheochromocytoma and paraganglioma: signs and symptoms related to catecholamine secretion

Minghao Li et al. Discov Oncol. .

Abstract

Background: The presence or future development of metastatic pheochromocytomas or paragangliomas (mPPGLs) can be difficult to diagnose or predict at initial presentation. Since production of catecholamines from mPPGLs is different from non-metastatic tumors (non-mPPGLs), this study aimed to clarify whether presenting catecholamine-related signs and symptoms (cSS) might also differ.

Methods: The study included 249 patients, 43 with mPPGL and 206 with non-mPPGL. Clinical data at the time of biochemical diagnosis (i.e. at entry into the study) were used to generate a cumulative score of cSS for each patient.

Results: Patients with mPPGL were significantly younger (43.3 ± 14 vs. 48.9 ± 16.1 years) and included a lower proportion of females (39.5% vs. 60.7%) than patients with non-mPPGLs. Frequencies of signs and symptoms did not differ between the two groups. Patients with mPPGLs had lower (P < 0.001) urinary excretion of epinephrine (3.5 (IQR, 1.9-6.5) µg/day) than those with non-mPPGLs (19.1 (IQR, 4.3-70.2) µg/day). There was no difference in urinary excretion of norepinephrine. In patients with mPPGLs a high cSS score was associated with high urinary excretion of norepinephrine and normetanephrine. In contrast, in patients with non-mPPGLs, a high cSS was associated with high urinary excretion of epinephrine and metanephrine.

Conclusion: Although presenting signs and symptoms were associated with production of norepinephrine in patients with mPPGLs and of epinephrine in patients with non-mPPGLs, there were no differences in signs and symptoms between the two groups. Therefore, consideration of signs and symptoms does not appear helpful for distinguishing patients with and without mPPGLs.

Keywords: Catecholamines; Metastatic; Paraganglioma; Pheochromocytoma; Signs; Symptoms.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Fig. 1
Fig. 1
Flow chart showing the patients included and excluded in this study. mPPGL, metastatic pheochromocytoma/paraganglioma; non-mPPGL, non-metastatic pheochromocytoma/paraganglioma. *Three of 14 developed metastatic disease more than one year after study entry
Fig. 2
Fig. 2
Urinary excretion of catecholamines and metanephrines in patients according to the cumulative score of signs and symptoms. Low low cumulative score of catecholamine related signs and symptoms, High high cumulative score of catecholamine related signs and symptoms, mPPGL metastatic pheochromocytoma/paraganglioma, non-mPPGL non-metastatic pheochromocytoma/paraganglioma
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References

    1. Turkova H, Prodanov T, Maly M, Martucci V, Adams K, Widimsky J, Jr, et al. Characteristics and outcomes of metastatic Sdhb and sporadic pheochromocytoma/paraganglioma: an national institutes of health study. Endocr Pract. 2016;22(3):302–314. doi: 10.4158/EP15725.OR. - DOI - PMC - PubMed
    1. Wang Y, Li M, Deng H, Pang Y, Liu L, Guan X. The systems of metastatic potential prediction in pheochromocytoma and paraganglioma. Am J Cancer Res. 2020;10(3):769–780. - PMC - PubMed
    1. Qin N, de Cubas AA, Garcia-Martin R, Richter S, Peitzsch M, Menschikowski M, et al. Opposing effects of HIF1alpha and HIF2alpha on chromaffin cell phenotypic features and tumor cell proliferation: insights from MYC-associated factor X. Int J Cancer. 2014;135(9):2054–2064. doi: 10.1002/ijc.28868. - DOI - PubMed
    1. Eisenhofer G, Lenders JW, Siegert G, Bornstein SR, Friberg P, Milosevic D, et al. Plasma methoxytyramine: a novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur J Cancer. 2012;48(11):1739–1749. doi: 10.1016/j.ejca.2011.07.016. - DOI - PMC - PubMed
    1. Gimenez-Roqueplo AP, Favier J, Rustin P, Rieubland C, Crespin M, Nau V, et al. Mutations in the SDHB gene are associated with extra-adrenal and/or malignant phaeochromocytomas. Cancer Res. 2003;63(17):5615–5621. - PubMed

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