. 2014 Jan 22;9(1):e86756. doi: 10.1371/journal.pone.0086756

Table 1. Clinical characteristics and carrier status.

	Cohort (n = 89)		No discovered mutations (n = 52)^*		Germline variants (n = 18)^**		Somatic variants (n = 16)		Germline and no mutation	Somatic and no mutation	Germline and Somatic mutation
		Range		Range		Range		Range	P
Median age, years	49,5	15–85	53	22–85	29,5	15–66	48	25–81	0.002^§	0.622^§	0.025^§
Gender, Male/Female	35/53		19/32		7/11		8/8		0.902^§§	0.365^§§	0.515^§§
Tumour specifications
Median size, mm	55	8–170	60	20–140	51 (n = 14)	20–170	45	8–100	0.166^§	0.305^§	0.755^§
Localization, left adrenal/right adrenal	31/37		22/21		3/5		5/9		0.478^§§	0.315^§§	0.933^§§
Adrenal/extra adrenal tumour	80/9		47/5		16/2		14/2		0.855^§§	0.740^§§	0.900^§§
Multifocal tumours	10		1		9		0		<0.001^§§	0.561^§§	<0.001^§§
Biochemistry
Median Urine Norepinephrine, nmol/24 h	1687	112–19130	2181,5	230–19130	836	112–4545	2439	225–14244	0.033^§	0.962^§	0.049^§
Median Urine Epinephrine, nmol/24 h	189	19–33322	247	19–33322	195	27–641	96	25–11664	0.2^§	0.541^§	0.622^§
Median Plasma Normetanephrine, nmol/L	6,4	1–37	7,1	4–16	1,4	1–2	11,6	1–37	0.004^§	0.640^§	0.136^§
Median Plasma Metaepinephrine, nmol/L	0,5	0–140	1,65	0–34	0,45	0–1	1,05	0–140	0.579^§	0.961^§	0.391^§
Recurrent disease	13		8		5		0		0.376^§§	0.070^§§	0.022^§§
Metastatic disease^***	9		7		2		0		0.797^§§	0.121^§§	0.169^§§

Statistical correlation of clinical variables and carriers status. Multifocal tumours was defined as bilateral pheochromocytoma or multiple paraganglioma tumours.

Patients with no available germline DNA were excluded.

^**

Included 14 germline variants and 4 patients with clinical criteria of Neurofibromatosis type 1.

^***

Nine patients had metastatic disease, one of these had metastatic disease at the time of diagnosis and the remaining had metastatic recurrences later on in the disease course. §Mann-Whitney U Test and §§Chi Square test.