Table 2.
Details of genetic mutation in all patients, primary tumor localization, grading (G) of tumor differentiation and mitotic index, as Ki-67, using MIB1 antibody. Additional secretion before PRRT and three months after PRRT.
| No | Gender | Age *, | Type of Mutation | Primary Tumor Localization | Grading (Ki-67 in %) |
Secretion before PRRT pg/mL | Secretion after PRRT pg/mL (Three Months) |
|---|---|---|---|---|---|---|---|
| 1 | M | 62 |
SDHD C11X,
ex 1 33TGC-TGC |
Right PPC | 2 (5) | normal | normal |
| 2 | F | 31 |
SDHD C11X,
ex 1 33TGC-TGC |
Chest & abdominal PGLs | 1 (2) | normal | normal |
| 3 | M | 52 |
SDHD C11X,
ex 1 33TGC-TGC |
Right HNP | 1 (1) | normal | normal |
| 4 | F | 51 |
SDHD C11X,
ex 1 33TGC-TGC |
Left HNP | 2 (3) | MTY = 1890 NMN = 126 |
MTY = 1060 NMN = 143 |
| 5 | M | 42 |
SDHD C11X,
ex 1 33TGC-TGC |
Right HNP | 1 (2) | NMN = 183,6 | NMN = normal |
| 6 | F | 47 |
SDHD C11X,
ex 1 33TGC-TGC |
Bilateral PPC | 1 (2) | normal | normal |
| 7 | M | 31 |
SDHD C11X,
ex 1 33TGC-TGC |
Left PPC | 1 (1) | normal | normal |
| 8 | F | 45 |
SDHD C11X,
ex 1 33TGC-TGC |
Left HNP | 1 (2) | normal | normal |
| 9 | M | 36 | SDHB ex.3 p.R90X | Left PPC | 1 (2) | MTY = 9189 NMN = 6911 |
MTY = 7235 NMN = 6430 |
| 10 | M | 38 | SDHB exon 1 deletion | Bladder PGL | 2 (5) | normal | normal |
| 11 | M | 27 |
SDHB c. 708 T > C
(int. 574 T > C) heterozygotic |
Paraspinal PGL | 2 (8) | MTY = 3344 NMN = 2622 |
MTY = 2570 NMN = 1987 |
| 12 | F | 39 | SDHB exon 1 deletion | Abdominal PGL | 2 (15) | normal | normal |
| 13 | M | 43 | SDHB R230L, exon7 | Left HNP | 2 (5) | normal | normal |
* Age of patients before PRRT SDHD-PGL1 syndrome, SDHB–PGL4 syndrome; PPC–pheochromocytoma, PGL–paraganglioma; HNP head and neck paraganglioma; MTY–Methoxytyramine; NMN–Normetanephrine in serum. Plasma NMN (pg/mL) upper reference intervals (age and gender adjusted): patient 4: 147; patient 5: 159.9; patient 9: 115; patient 11: 106; Plasma MN (pg/mL): 88, Plasma MTY (pg/mL): 30.