Tab. I.
Patient no. | Gender | Age [years] | Gene | Mutation type | Thoracic PGL found at initial diagnosis | Tumor location |
Tumor size [cm] on CT |
Surgical resection | Multifocal PGLs |
SRS 99mTc [TOC] SPECT |
123I mIBG* SPECT | Malignant |
---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | F | 48 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Posterior mediastinum Th2–Th3 |
2 × 2.3 | Yes | Yes HNP, pheo, abdomen extraadrenal |
Positive | Positive | No |
2 | F | 43 | SDHD | Exon 1 deletion | No | Middle mediastinum (between LA, right pulmonary artery, ascending aorta) | 4.5 × 3.5 | Yes | Yes Abdomen Extraadrenal |
Positive | Positive | No |
3 | M | 30 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (between aortic root, pulmonary trunk and LAD) |
1.6 × 1.3 | No | Yes HNP, pheo |
Positive | Negative | No |
4 | F | 39 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (between aortic root, pulmonary trunk and LAD) |
1.3 × 1.2 | No | Yes HNP, pheo, Abdomen Extraadrenal |
Positive | Negative | No |
5 | M | 59 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (aortopulmonary window) |
2.1 × 1.8 | No | Yes HNP |
Positive | Negative | No |
6 | F | 40 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (between LA and descending aorta) | 3.7 × 2.0 | Yes | Yes HNP, abdomen extraadrenal |
Positive | ND | No |
7 | F | 34 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (between right pulmonary artery and LA) Middle mediastinum (aortopulmonary window) |
1 × 1.1 2.7 × 3.5 |
Yes Yes |
Yes HNP, abdomen extraadrenal |
Positive Positive |
Negative Negative |
No |
8 | M | 33 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (aortopulmonary window) | 2.5 × 1.7 | No | Yes HNP, pheo |
Positive | Negative | No |
9 | M | 64 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (between VCS, ascending aorta, right pulmonary artery and trachea) | 3 × 2 | Yes | Yes HNP |
ND | ND | No |
10 | M | 28 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (aortic pulmonary window) | 1.5 × 1.6 | No | Yes HNP |
Positive | Negative | No |
11 | M | 26 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (right atrioventricular groove near RCA) |
3.6 × 2.3 | No | Yes HNP |
Positive | Negative | No |
12 | F | 30 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Posterior mediastinum | N | Yes | Yes HNP, abdomen extraadrenal |
ND | ND | No |
13 | F | 33 | SDHD | Exon 1, c.33C > A, p. C11X |
No | Middle mediastinum (aortopulmonarywindow) | 0.7 × 0.6 | No | Yes HNP |
Negative | Negative | No |
14 | F | 52 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
?? | Middle mediastinum (between aortic arch, right pulmonary artery, VCS, trachea) | 5.2 × 4.7 | Yes | Yes HNP |
ND | ND | No |
15 | M | 22 | SDHB | Exon 6, c.708T > C (int. 574 T > C), p. C192R Missense |
Yes | Posterior mediastinum Th8–Th11 | N | Yes | Yes HNP |
Positive | Positive | Yes |
16 | M | 40 | SDHD | Exon 1, c.33C > A, p. C11X Nonsense |
No | Middle mediastinum (between aortic bulb, LAD and LA) |
3.5 × 2 | No | Yes HNP |
ND | ND | No |
ND – not done, VCS – venae cave superior, LA – left atrium, LAD – left descending artery, RCA – right coronary artery, HNP – head and neck paraganglioma, pheo – pheochromocytoma, N – no data are available, only histological outcome