Table 2 Clinical, pathologic, and IHC features of 62 previously unreported SDH-deficient RCCs from 59 patients.
Patient no. | Location | Age | Sex | Size (cm) | Surgery | Necrosis | Mitotic figures (per 2mm2) | T stage | WHO/ISUP nucleolar (nuclear) grade | Status | Duration of follow up (months) | Germline Mutation | SDHB | SDHA | PAX8 | AMACR | KIT | CK7 | AE1/AE3 |
|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | Right | 22 | F | 10 | Partial nephrectomy | No | 1 | 3c | 3 | DOD | 27 | SDHB [c.505 C > T,p.Gln169] | Neg | Pos | Pos | U | Neg | Neg | U |
Right | 22 | F | 4.5 | Completion nephrectomy | No | 4 | 3c | 4a | DOD | 27 | Neg | Pos | U | U | U | U | U | ||
2 | Right | 64 | M | 6.5 | Partial nephrectomy | No | 0 | 1b | 1 | U | 27 | SDHB [c.72 + 1 G > T] | Neg | Pos | Pos | U | Neg | Neg | U |
3 | Right | 52 | M | 6.5 | Nephrectomy | No | 0 | 1b | 1 | U | U | Neg | Pos | U | U | U | U | U | |
4 | Left | 56 | M | 8 | Nephrectomy | No | 0 | 2a | 2 | U | U | SDHB [c.557 G > A, p.Cys186Tyr] | Neg | Pos | Pos | Pos | Neg | Neg | U |
5 | Right | 32 | M | 9.5 | Nephrectomy | No | 1 | 2a | 3 | U | U | SDHB [C.268 C > T,p.Arg90] | Neg | Pos | Pos | Pos | Neg | Neg | U |
6 | Left | 54 | F | 2.5 | Partial Nephrectomy | No | 0 | 1a | 2 | A | 62 | SDHB [c.725 G > A, p.Arg242His inv exon 7] | Neg | Pos | Pos | U | U | U | Pos |
7 | U | U | M | U | U | No | 0 | U | 2 | U | U | Neg | Pos | U | U | U | U | U | |
8 | Right | 22 | M | 1 | Nephrectomy | No | 0 | 1a | 2 | U | U | SDHB [c.293 G > C, p.(Cys98Ser)] | Neg | Pos | U | U | U | U | U |
9 | Right | 51 | M | 6.5 | Nephrectomy | No | 0 | 1b | 3a | U | U | Neg | Pos | U | U | U | U | Pos | |
10 | Left | 19 | M | 6 | Partial nephrectomy | No | 1 | 1b | 3a | U | U | Neg | Pos | U | Pos | Neg | Neg | U | |
11b | Right | 48 | M | 9 | Partial nephrectomy | No | 3 | 2a | 4a | U | U | Neg | Pos | U | U | Neg | Neg | U | |
Left | U | M | 9 | Partial Nephrectomy | No | 2 | 2a | 2 | U | U | Neg | Pos | U | U | U | U | U | ||
12 | Right | 47 | F | 5 | Partial nephrectomy | No | 1 | 1b | 2 | A | 27 | SDHB exon 8 deletion | Neg | Pos | U | U | U | U | U |
13 | Right | 20 | F | 4.8 | Partial nephrectomy | No | 4 | 1b | 2 | U | U | Neg | Pos | U | U | U | U | U | |
14 | Left | 68 | M | 3.8 | Nephrectomy | No | 6 | 1a | 2 | U | U | Neg | Pos | U | U | U | U | U | |
15 | Left | 61 | F | 15 | Nephrectomy | Yes | 1 | 3b | 3 | AWM | 10 | Neg | Pos | U | U | U | U | U | |
16 | Left | 21 | F | 15 | Nephrectomy | No | 0 | 3 | 3 | U | U | Neg | Pos | U | U | U | Neg | U | |
17 | Right | 26 | F | 4 | Partial nephrectomy | No | 0 | 1b | 2 | U | U | Neg | Pos | Pos | U | Neg | Neg | Neg | |
18 | Left | 38 | F | 5.5 | Partial nephrectomy | No | 0 | 1b | 2 | AWD | 16 | Neg | Pos | Pos | U | Neg | Neg | U | |
19 | Right | 25 | M | 2 | Partial nephrectomy | No | 0 | 1a | 2 | A | 3 | SDHB germline mutation | Neg | Pos | U | U | U | U | U |
20b | Left | 22 | F | 13 | Nephrectomy | No | 0 | 2b | 2 | A | 57 | Neg | Pos | U | U | Neg | U | U | |
21 | Right | 30 | F | 8.5 | Nephrectomy | No | 0 | 2a | 2 | U | U | SDHB [c.137 G > A] | Neg | Pos | U | Neg | Pos | Neg | U |
22 | Left | 25 | M | 5.8 | Nephrectomy | No | 0 | 1b | 3 | A | 13 | SDHB exon 3 deletion | Neg | Pos | U | U | U | U | U |
23 | U | U | U | U | U | No | 0 | U | 2 | U | U | Neg | Pos | U | U | U | U | U | |
24 | Left | 50 | M | 7.2 | Nephrectomy | Yes | 2 | 2a | 3 | DOD | 24 | SDHB [c.272 G > T,p.Arg91Ile] | Neg | Pos | Pos | Pos | Neg | Pos | Pos |
25 | Bilateral and multifocal | 72 | F | 5 | Partial nephrectomy | No | 0 | 1b | 1 | AWD | 72 | Neg | Pos | Pos | Pos | Pos | Neg | U | |
26 | U | 28 | F | 7.9 | Partial nephrectomy | No | 0 | 2a | 3 | A | 7 | SDHB germline mutation | Neg | Pos | Pos | U | Neg | Neg | U |
27 | Left | 58 | F | 20 | Nephrectomy | No | 10 | 3a | 3 | AWD | 6.21 | Neg | Pos | U | U | Neg | Neg | Pos | |
28 | Left | 29 | M | 8.5 | Nephrectomy | No | 0 | 2a | 2 | AWD | 6 | Neg | Pos | U | U | U | U | U | |
29 | Right | 44 | M | 22 | Nephrectomy | No | 0 | 2b | 2 | DOD | 26 | Neg | Pos | U | U | Neg | U | U | |
30 | Left | 37 | M | 14.5 | Nephrectomy | Yes | 1 | 3a | 2 | AWD | U | Neg | Pos | U | U | U | U | U | |
Left | 37 | M | 7 | Nephrectomy | Yes | 0 | 1b | 4a | AWD | U | Neg | Pos | U | U | U | U | U | ||
31 | U | 45 | M | 3.5 | Nephrectomy | No | 0 | 1a | 2 | U | U | Neg | Pos | U | U | U | U | U | |
32 | Left | 29 | M | 0.5 | Nephrectomy | No | 0 | 1a | 2 | U | U | Neg | Pos | U | U | U | U | U | |
33 | Left | 30 | M | 2 | Partial nephrectomy | No | 0 | 1a | 2 | U | 60 | SDHB germline mutation | Neg | Pos | U | U | U | U | U |
34 | Left | 55 | M | 2.5 | Partial nephrectomy | No | 0 | 1a | 2 | U | U | Neg | Pos | U | U | U | U | U | |
35 | Left | 33 | M | 1.7 | U | Yes | 3 | 1a | 3a | AWM | U | Neg | Pos | Pos | Pos | U | Neg | U | |
36 | Right | 27 | M | 8.2 | Nephrectomy | Yes | 1 | 2a | 3 | AWM | 50 | SDHB germline mutation | Neg | Pos | U | Pos | U | Neg | U |
37 | Right | 80 | M | 10 | Nephrectomy | Yes | 1 | 3a | 2 | A | 2 | Neg | Pos | Pos | U | Neg | Neg | Pos | |
38 | Right | 57 | M | 10.4 | Nephrectomy | Yes | 1 | 3a | 3 | A | 0.5 | Neg | Pos | U | U | Neg | Neg | Neg | |
39 | Left | 22 | M | 11 | Nephrectomy | No | 1 | 2b | 2 | A | U | Neg | Pos | Pos | Pos | Neg | Neg | Pos | |
40 | U | 25 | M | 7 | Nephrectomy | Yes | 0 | 1b | 4a | U | U | Neg | Pos | U | U | Neg | Pos | U | |
41 | Left | 22 | F | 2.2 | Partial nephrectomy | No | 0 | 1a | 2 | A | 60 | SDHB germline mutation | Neg | Pos | Neg | Pos | Neg | Neg | Neg |
42 | U | 56 | M | 3.5 | Partial nephrectomy | No | 0 | 1a | 2 | A | 11 | Neg | Pos | Pos | U | Neg | Neg | U | |
43 | Right | 35 | F | 13 | Nephrectomy | No | 10 | 2b | 4a | AWM | 103 | SDHB [R46] | Neg | Pos | Pos | U | U | U | U |
44 | U | 33 | F | 10 | Nephrectomy | Yes | 1 | 3a | 3a | AWD | 12 | Neg | Pos | Pos | Pos | Pos | Neg | Pos | |
45 | Left | 39 | M | 3.5 | Partial nephrectomy | No | 0 | 1a | 2 | A | 3 | SDHB germline mutation | Neg | Pos | U | U | U | U | U |
46 | Left | 53 | F | 6.5 | Nephrectomy | No | 1 | 1b | 2 | A | 120 | Neg | Pos | U | U | U | U | U | |
47 | Right | 76 | M | 2.6 | Partial nephrectomy | No | 1 | 1a | 2 | DOUC | 3.5 | Neg | Pos | U | U | U | U | U | |
48 | Right | 39 | M | 5.2 | Partial nephrectomy | No | 0 | 1b | 1 | A | 0.5 | Neg | Pos | U | U | Neg | Neg | Neg | |
49 | Left | 57 | F | 1.4 | Partial nephrectomy | No | 0 | 1a | 3 | A | 60 | Neg | Pos | Neg | U | Neg | Neg | Neg | |
50 | Left | 46 | M | 7 | Nephrectomy | No | 0 | 1a | 2 | A | 1 | Neg | Pos | Pos | U | Neg | U | U | |
51 | Right | 69 | F | 7 | Nephrectomy | No | 1 | 1b | 2 | A | 1 | Neg | Pos | Pos | U | Pos | U | U | |
52 | Right | 23 | M | 11 | Brain metastectomy | No | 1 | U | 3a | AWM | 4 | Neg | Pos | U | U | U | Neg | Neg | |
53 | Left | 49 | M | 9.4 | Nephrectomy | No | 0 | 2a | 2 | A | 1 | Neg | Pos | U | U | U | Neg | U | |
54 | Left | 66 | F | 4.5 | Nephrectomy | Yes | 1 | 4 | 3a | A | 1 | Neg | Pos | U | U | U | U | U | |
55 | Left | 58 | F | 3.8 | Partial nephrectomy | No | 0 | 1a | 2 | A | 0.5 | Neg | Pos | U | U | Neg | Neg | U | |
56 | Right | 51 | M | 8.4 | Partial nephrectomy | No | 0 | 2a | 2 | A | 3 | SDHB splice site variant | Neg | Pos | U | Neg | Neg | Neg | U |
57 | Right | 43 | F | 2.9 | Partial nephrectomy | No | 0 | 1a | 2 | A | 6 | Neg | Pos | Pos | Pos | Neg | Neg | U | |
58 | Right | 29 | M | 12 | Nephrectomy | Yes | 0 | 3a | 3a | A | 1 | Neg | Pos | Pos | Pos | U | Pos | U | |
59 | Right | 33 | M | 13.3 | Core biopsy | Yes | 10 | 4 | 4a | AWD | 1 | Neg | Pos | Pos | Neg | Neg | Neg | U |
