Table 2.
PC and PGL Phenotype in PGL3 Patients
| Study | Year | No. of Patients | No. of Tumors | HNPGL | Thoracic | Abdomen | Adrenal | Single | Multiple | Malignant |
|---|---|---|---|---|---|---|---|---|---|---|
| Niemann et al (1, 2) | 2000 | 5 | 6 | 5 | 0 | 0 | 0 | 4 | 1 | 1 |
| Niemann et al (3) | 2003 | 1 | 1 | 1 | 0 | 0 | 0 | 1 | 0 | 0 |
| Bauters et al (4) | 2003 | 2 | 2 | 1 | 1 | 0 | 0 | 2 | 0 | 0 |
| Baysal et al (5) | 2004 | 6 | 7 | 7 | 0 | 0 | 0 | 5 | 1 | 0 |
| Schiavi et al (6) | 2005 | 8 | 8 | 8 | 0 | 0 | 0 | 8 | 0 | 0 |
| Bayley et al (7) | 2006 | 1 | 1 | 1 | 0 | 0 | 0 | 1 | 0 | 0 |
| Peczkowska et al (8) | 2007 | 2 | 2 | 1 | 0 | 0 | 1 | 2 | 0 | 0 |
| Zbuk et al (9) | 2007 | 1 | 1 | 1 | 0 | 0 | 0 | 1 | 0 | 0 |
| Lopez-Jiminez et al (10) | 2008 | 1 | 1 | 1 | 0 | 0 | 0 | 1 | 0 | 0 |
| Pasini et al (11) | 2008 | 2 | 2 | 0 | 0 | 2 | 0 | 2 | 0 | 0 |
| Burnichon et al (12)a | 2009 | 16 | 21 | 18 | 3 | 0 | 0 | 11 | 5 | 0 |
| Bayley et al (13) | 2009 | 1 | 1 | 1 | 0 | 0 | 0 | 1 | 0 | 0 |
| Papaspyrou et al (14) | 2011 | 7 | 13 | 10 | 0 | 0 | 0 | 4 | 3 | 1 |
| Illouz et al (15) | 2012 | 1 | 1 | 0 | 1 | 0 | 0 | 1 | 0 | 0 |
| Present study, Else et al | 2012 | 8 | 12 | 9 | 3 | 0 | 0 | 4 | 4 | 0 |
| Total n | 62 | 79 | 64 | 8 | 2 | 1 | 48 | 14 | 2 | |
| Total % | 81.0%a | 10.1%a | 2.5%a | 1.3%a | 77.4%b | 22.6%b | 2.5%a |
Mean age, 38.4 years (51 available); catecholamine production of 34 available: 8 (23.5%) positive (1 adrenal, 4 thoracic, 3 HNPGL), and 26 (76.5%) negative (all HNPGL). References: please see supplemental material.
a
Percent of all tumors.
b
Percent of all patients.