TY  - JOUR
A1  - Li, Minghao
A1  - Pamporaki, Christina
A1  - Fliedner, Stephanie M. J.
A1  - Timmers, Henri J. L. M.
A1  - Nölting, Svenja
A1  - Beuschlein, Felix
A1  - Prejbisz, Aleksander
A1  - Remde, Hanna
A1  - Robledo, Mercedes
A1  - Bornstein, Stefan R.
A1  - Lenders, Jacques W. M.
A1  - Eisenhofer, Graeme
A1  - Bechmann, Nicole
T1  - Metastatic pheochromocytoma and paraganglioma: signs and symptoms related to catecholamine secretion
JF  - Discover Oncology
N2  - 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.
KW  - pheochromocytoma
KW  - paraganglioma
KW  - metastatic
KW  - signs
KW  - symptoms
KW  - catecholamines
Y1  - 2021
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-309901
SN  - 2730-6011
VL  - 12
ER  -