Figure 1.
Figure 2.
Figure 3.
First-line systemic treatment regimens for inoperable locally advanced or metastatic disease
| Treatment regimen | Patients (N) |
|---|---|
| EDP-mitotane | 11 |
| mitotane (+/- local therapy) | 11 |
| etoposide + carboplatin | 1 |
| dacarbazine + cyclophosphamide + vincristine | 1 |
| tamoxifen | 1 |
Ki67 proliferation index and Helsinki score shown for primary tumour (P) and first metastasis/local recurrence (M)
| Patient | Ki67 | Helsinki score | ||
|---|---|---|---|---|
| P | M | P | M | |
| 1 | 16 | 40 | 24 | 48 |
| 2 | 20 | 16 | 23 | 24 |
| 3 | 15 | 25 | 23 | 33 |
| 4 | 20 | 20 | 28 | 23 |
| 5 | 30 | 30 | 38 | 38 |
| 6 | 40 | 30 | 48 | 38 |
| 7 | 15 | 11 | 18 | 19 |
| 8 | 30 | 20 | 38 | 28 |
| 9 | 10 | 11 | 18 | 19 |
| 10 | 40 | 50 | 48 | 50 |
| 11 | 25 | 50 | 29 | 58 |
Characteristics of all analysed patients and of the patients with European Network for the Study of Adrenal Tumors (ENSAT) I-III that relapsed after surgery with curative intent
| Characteristics | All included N = 48 (%) | Relapsed N = 20 (%) | |
|---|---|---|---|
| Age: median (range); years | 56.6 (21–82) | 54.0 (21–72) | |
| Sex | Male | 21 (44) | 11 (55) |
| Female | 27 (56) | 9 (45) | |
| ENSAT stage at diagnosis | I | 3 (6) | 0 |
| II | 20 (42) | 12 (60) | |
| III | 12 (25) | 8 (40) | |
| IV | 13 (27) | N/R | |
| Tumour size: median (range), cm | 12 (4–30) | 12.5 (5–30) | |
| Unknown | 6 | 2 | |
| Hormone secretion | Yes – GC* | 17 (35) | 5 (25) |
| Yes – O | 8 (17) | 4 (20) | |
| No | 20 (42) | 11 (55) | |
| Unknown | 3 (6) | / | |
| Weiss score (median, range) | 6 (4–9) | 7 (5–9) | |
| N/D+ | 15 | 1 | |
| Ki67 score** (median, range) | 20 (1–70) | 24 (8–60) | |
| N/D° | 9 | 1 | |
| Helsinki score** (median, range) | 28 (1–78) | 31 (16–68) | |
| N/D° | 9 | 1 | |
| Resection margins of patients | RO | 26 (76) | 17 (85) |
| stage I –III treated with curative | R1 | 5 (15) | 3 (15) |
| surgery | Rx | 3 (9) | / |
Second line treatment regimens
| Treatment | Patients (N) | Response |
|---|---|---|
| gemcitabine + capecitabine +/- mitotane | 5 | SD: 1 |
| EDP-mitotane | 1 | PR |
| pembrolizumab | 1 | SD |
| dacarbazine + capecitabine + imatinib | 1 | PD |
| vinblastine + interferon alpha-2a | 1 | PD |