Table 1.

Per lesion and per patient detection rates of ⁶⁸Ga-DOTA-SSA PET/CT with ¹⁸F-FDOPA PET/CT and/or ¹⁸F-FDG PET/CT and/or anatomic imaging in various cohorts of PPGL

Cohort	Authors/Year	Country	Type of study	Gold Standard	No. of PPGL patients	68Ga-DOTA-SSA		18F-DOPA		18F-FDG		CT/MRI
					(n)	Per patient	Per lesion	Per patient	Per lesion	Per patient	Per lesion	Per patient	Per lesion
Metastatic SDHB-related PPGL	Janssen, et al. 2015	USA	Prospective single center	Composite of anatomic and functional imaging	17	17/17 (100%)	285/289 (98.6%)	14/16 (87.5%)	175/285 (61.4%)	17/17 (100%)	248/289 (85.8%)	17/17 (100%)	245/289 (84.8%)
						n=17	n=17	n=17	n=16	n=17	n=17	n=17	n=17
Sporadic metastatic PPGL	Janssen, et al.2016	USA	Prospective single center	Composite of anatomic and functional imaging	22	22/22 (100%)	450/461 (97.6%)	11/12 (91.7%)	181/242 (74.8%)	20/22 (90.9%)	226/461 (49.2%)	22/22 (100%)	376/461 (81.6%)
						n=22	n=22	n=22	n=12	n=22	n=22	n=22	n=22
Pediatric SDHx-related PPGL	Jha, et al.2018	USA	Retrospective single center	Composite of anatomic and functional imaging	9	8/8 (100%)	100/107 (93.5%)	N.A.		8/8 (100%)	85/107 (79.4%)	8/8 (100%)	79/107 (73.8%)
						n=8	n=8			n=8	n=8	n=8	n=8
Head and neck PGL	Janssen, et al.2016	USA	Prospective single center	18F-FDOPA or CT/MRI	20	N.A.	38/38 (100%)	N.A.	37/38 (97.4%)	N.A.	27/38 (71.1%)	N.A.	23/38 (60.5%)
							n=20		n=20		n=20		n=20
Polycythemia-PGL syndrome	Janssen, et al. 2017	USA	Prospective single center	Composite of anatomic and functional imaging	14	N.A.	24/68 (35.3%)	N.A.	73/74 (98.7%)	N.A.	22/52 (42.3%)	N.A.	44/74 (60.3%)
							n=13		n=14		n=13		n=14
MAX-related PPGL	Taieb, et al. 2018	USA, France	Retrospective bicentric	Histology	6	3/3 (100%)	4/7 (57.1%)	6/6 (100%)	20/22 (90.9%)	2/4 (50.0%)	2/11 (18.2%)	5/6 (85.7%)	11/21 (52.4%)
						n=3	n=3	n=3	n=6	n=4	n=4	n=6	n=6
Mixed cohort	Archier et al. 2016	France	Prospective single center	Histology and composite of anatomic and functional imaging	30	28/30 (93%)	43/46 (93%)	29/30 (97%)	41/46 (89%)	N.A.		28/30 (93%)	35/46 (76%)
						n=30	n=29	n=30	n=29			n=30	n=29
Extraadrenal PGL	Kroiss et al. 2013	Austria	Retrospective single center	Combined cross-sectional imaging	20	20/20 (100%)	45/45 (100%)	20/20 (100%)	32/45 (71.1%)	N.A.		N.A.
						n=20	n=20	n=20	n=20

^*:The study reports mean number of ⁹⁰Y-DOTATATE PRRT cycles per patient and does not provide the range of cycles. CT/MRI: anatomic imaging with computed tomography and/or magnetic resonance imaging; ¹⁸F-FDG: ¹⁸F-fluorodeoxyglucose; ¹⁸F-FDOPA: ¹⁸F-fluorodihydroxyphenylalanine; ⁶⁸Ga-DOTA-SSA: gallium-68 (⁶⁸Ga)-labeled somatostatin receptor analogs; MAX: germline mutation of MYC-associated factor X gene; N.A.: not available; PGL: paraganglioma; PPGL: pheochromocytoma/paraganglioma; PET/CT: positron emission tomography-computed tomography; SDHB: germline mutation of succinate dehydrogenase subunit B gene; SDHx: SDHx: germline mutation in one of the succinate dehydrogenase complex genes.

Note: Includes the studies where head-to-head comparison between ⁶⁸Ga-DOTA-SSA PET/CT and ¹⁸F-FDOPA PET/CT was performed. The data from or ¹⁸F-FDG PET/CT and/or anatomic imaging was also included if they were available. The reference standard or denominator was heterogeneous among various studies and ranged from histology to composite of anatomical and functional imaging. Even though pathology is considered as gold standard, in patients with metastatic disease biopsy of multiple sites is neither feasible nor ethical. Therefore, adoption of a composite of imaging and/or pathologic findings should be considered a robust alternative (Hofman and Hicks et al. 2015).