Table 1.

Clinical characteristics of patients from whom the PPGLs resected.

Total PPGLs	65
Total no. of patients	61
Gender
Male (n (%))	43 (71%)
Female (n (%))	18 (29%)
Age at diagnosis (years) (mean (range))	40 (14–80)
Duration of FU (years) (mean (range))	7.8 (2–26)
Size of tumour (mm) (mean (range))	55.2 (10–235)
PCC (n = 38)	49.1 (10–114)
Abdo PGL (n = 17)	64 (19–235)
Thorax PGL (n = 5)	70.4 (45–117)
Pelvic PGL (n = 3)	41 (30–52)
HNPGL (n = 2)	70 (30–110)
No. of primary PPGLs (n (%))	56 (86%)
No. of multiple PGLs* (n (%))	4 (6%)
No. of recurrent PGLs (n ([%))	5 (8%)
Functional
Yes	49 (75.3%)
No	8 (12.3%)
Unknown	8 (12.3%)
Raised MN > ULN (n (%))	21 (32%)
Raised NMA > ULN (n (%))	30 (46%)
Raised 3MT > ULN (n (%))	5 (2%)
Urine metanephrine levels (nmol) (n (%))	44 (67.7%)
Metanepherine (mean ± s.e.m.) NR < 2000)	11,122 ± 2840
Normetanepherine (mean ± s.e.m.) (NR < 4400)	22,760 ± 4930
3MT (mean ± s.e.m.) (NR < 2500)	2775 ± 478
Plasma metanephrine levels (pmol/L) (n (%))	23 (35.4%)
Metanepherine (mean ± s.e.m.) (NR < 510)	3475 ± 1175
Normetanepherine (mean ± s.e.m.) (NR < 1180)	9814 ± 2421
3MT (mean ± s.e.m.) (NR < 180)	155 ± 71.3
Aggressive (n (%))	15+ (23%)
Indolent (n (%))	50 (77%)
Genetics tested (n (%))	54 (83%)
Positive (n (%))	33 (50%)
Negative (n (%))	21 (32%)
SDHB	20 (8*) (40%)
RET	6 (9%)
VHL	4 (6%)
SDHA	2 (1*) (3%)
FH	1 (1.5%)
Negative	21 (2*) (32.3%)
Not tested (NT)	11 (3*) (20%)

*Denotes number of tumours in category that were aggressive in nature. ⁺Includes 13 PPGLs that were metastatic and 2 PPGLs that were locally invasive. FU, follow up; HNPGL, head and neck paraganglioma; MN, metanepherine; NMA, normetanepherine; NR, normal range; PCC, phaeochromocytoma; PGL, paraganglioma; 3MT 3-methyoxytyramine.