Table 1.

Patient characteristics

	Gender, agea (years)	F/U (years)	Mutation	Biochemistry (fold normal)	Presenting symptoms	Primary mediastinal tumor	Other PGL/Pheo	Therapy	Outcome
1	F, 18	7	SDHB, R242H	NM (4×)	Hypertension, palpitations, chest pain	T4 vertebral body (2.5 cm), recurrent disease	No	Thoracotomy, XRT	Five years to meta spine, psoas
2	F, 60	1	SDHB, G96D	NM (11×)	Chest pain, upper back pain, weight loss, hypertension	T5–T7 vertebral body (7.5 cm), epidural space, thoracic wall, paraspinal musculature	No	Surgical removal	Stable disease
3	M, 25	7	SDHB, R46X	Normal	Chest pain	Posterior mediastinal mass, left paraspinal T2–T3 (3.5 cm)	No	Surgery paraspinal mass, XRT lobectomy, thoracic corpectomy, decompression, fixation T1–T5	Four years to meta lung, superior mediastinum, spine, sternum, scapula 6 years to meta left ischium, T3 and T4
4	M, 45	9	SDHD, c.170-1 G>T	NM (2.6×)	Weakness, palpitations, headaches, hypertension	Middle mediastinal mass (6 cm) with compression right main pulmonary artery	HNP	Embolization, partial resection	One year to recurrent disease, meta lung, subcarinal lesion, proximal tibia
5	M, 26	4	SDHB, R46Q	Normal	Low back pain, hypertension	Left superior mediastinum (15.6 cm) right superior paratracheal region inferior right paratracheal lymph nodes	PGL abdomen	Surgery abdominal mass six cycles etoposide/-cisplatin octreotide	Necrotic mass L4, extending in pelvis, 1 year to meta lesions L3 and L4 and lymph nodes
6	M, 19	19	SDHD, L20C fsX66	NM (2×)	Hemodynamic instability during HNP surgery	Paratracheal mass (2 cm)	HNP Pheo	Carotid body removal right, adrenalectomy right, XRT (HNP), 12 cycles CVD, 131I-MIBG-therapy	Fifteen years to recurrence to mediastinum. Meta left ilium
7	F, 36	6	SDHB, R46X	NM (3.5×) DA (1.9×)	Chest discomfort, hypertension, palpitations, pre-syncope associated with urination	Posterior mediastinal mass next to esophagus, posterior wall left atrium (2.9 cm)	PGL bladder	Partial cystectomy, re-operation (negative), surgery mediastinal PGL	Stable disease
8	F, 50	6	SDHD, P81L	Normal	Upper respiratory symptoms, new-onset hypertension	Aorto-pulmonary window, left and right hilus (3.3 cm), pulmonary nodules	HNP	Pending	Meta lung, mediastinum, 6 years later being evaluated for new-onset hypertension
9	M, 41	1	SDHD, D92Y	VMA (1.3×)b DA (1.1×)b	Excessive perspiration	Two masses in aorto-pulmonary window (1 cm)	HNP	Watch-and-wait	Stable disease
10	M, 18	1	SDHB, c.286+2 T>A	NM (10×)	Hypertension	Mass in posterior mediastinum (9 cm), growth into T5	No	Surgery (incomplete)	Stable disease
Mean (range)	34 (18–60)					Primary tumor: 5.3 cm (1–15.6)

F/U, follow-up; NM, normetanephrine; DA, dopamine; VMA, vanillyl mandelic acid; T, thoracic; L, lumbar; XRT, radiotherapy; Meta, metastasis; CVD, dacarbazine; HNP, head and neck paraganglioma; PGL, paraganglioma; Pheo, pheochromocytoma.

^aAge at diagnosis.

^bUrine measurement.