Demographic, clinical, and histopathological characteristics of 34 patients with primary ACC and results of ccfDNA analysis.
| Patient-ID . | Sex/age . | Symptoms . | ENSAT tumour stage . | Metastasis at diagnosis . | ccfDNA levels (ng/µL of plasma) . | ccfDNA sequencing - Gene name . | Surgery . | R status . | Ki67 index . | S-GRAS score . | Available Follow-up . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| ACC-P1 | F/55 | Yes | 4 | Liver, lung, LN | 0.195 | CTNNB1(NM_001098209.1) | No | NA | NA | NA | No |
| ACC-P2 | M/83 | Yes | 3 | None | 0.301 | No variants identified | Yes | 0 | 9 | 3 | Long |
| ACC-P3 | M/43 | Yes | 3 | None | Yes | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 0 | 30 | 4 | Long |
| ACC-P4 | M/71 | No | 3 | None | 0.173 | No variants identified | Yes | 0 | 40 | 4 | Short |
| ACC-P5 | F/23 | Yes | 4 | Lung | 1.935 | No variants identified | Yes | 2 | 40 | 8 | Short |
| ACC-P6 | M/36 | Yes | 2 | None | 0.151 | No variants identified | Yes | 0 | 3 | 1 | Long |
| ACC-P7 | F/68 | Yes | 4 | Liver | 2.325 | No variants identified | Yes | 2 | 30 | 9 | No |
| ACC-P8 | F/74 | No | 3 | None | 1.120 | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 1 | 40 | 6 | Short |
| ACC-P9 | M/37 | Yes | 4 | Liver, lung | 0.735 | TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P10 | F/54 | Yes | 4 | Lung | 2.700 | ZNRF3 (NM_001206998.1) | No | NA | NA | NA | No |
| ACC-P11 | M/64 | Yes | 4 | Liver | 4.350 | CTNNB1 (NM_001098209.1) DAXX (NM_001141970.1 RB1 (NM_000321.2) TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P12 | F/73 | Yes | 4 | Liver | 6.000 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P13 | M/57 | Yes | 4 | Liver | 0.401 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P14 | F/36 | Yes | 3 | None | 0.193 | No variants identified | Yes | 1 | 30 | 6 | Short |
| ACC-P15 | F/57 | Yes | 3 | None | 0.890 | No variants identified | Yes | 1 | 10 | 6 | Long |
| ACC-P16 | M/50 | No | 2 | None | 0.062 | NF1 (NM_000267.3)b | Yes | 0 | 17 | 1 | Short |
| ACC-P17 | F/65 | Yes | 3 | None | 0.385 | GNAS (NM_000516.5) | Yes | 1 | 25 | 7 | Short |
| ACC-P18 | F/60 | Yes | 4 | Lung, LN | 5.500 | CTNNB1 (NM_001098209.1) ATM (NM_000051.3)b | No | NA | NA | NA | No |
| ACC-P21 | F/56 | No | 2 | None | 0.251 | No variants identified | Yes | 0 | 30 | 3 | Long |
| ACC-P22 | F/50 | No | 3 | None | 0.247 | No variants identified | Yes | 0 | 18 | 3 | Long |
| ACC-P23 | F/27 | Yes | 3 | None | 0.189 | No variants identified | Yes | 0 | 40 | 4 | No |
| ACC-P24 | M/31 | Yes | 2 | None | 0.163 | No variants identified | Yes | 0 | 12 | 2 | Long |
| ACC-P25 | F/56 | No | 3 | None | 0.454 | No variants identified | Yes | 0 | 5 | 2 | Long |
| ACC-P26 | F/39 | No | 2 | None | 0.185 | CTNNB1 (NM_001098209.1) | Yes | 0 | 40 | 2 | Short |
| ACC-P27 | M/64 | No | 1 | None | 0.114 | No variants identified | Yes | 0 | 23 | 3 | No |
| ACC-P28 | F/35 | Yes | 4 | Lung | 1.473 | TERT (NM_198253.2) (NM_002734.4) | Yes | 2 | 80 | 8 | Short |
| ACC-P29 | F/56 | Yes | 3 | None | 0.302 | No variants identified | Yes | 0 | 12 | 4 | Short |
| ACC-P30 | F/53 | Yes | 2 | None | 0.368 | APC (NM_000038.5) | Yes | 0 | 30 | 4 | Long |
| ACC-P31 | F/51 | Yes | 2 | None | 1.380 | ATM (NM_000051.3)b | Yes | 0 | 22 | 4 | Long |
| ACC-P32 | F/26 | Yes | 4 | Lung | 0.414 | No variants identified | No | NA | 19a | NA | No |
| ACC-P33 | M/48 | Yes | 4 | Liver, lung | 0.846 | No variants identified | No | NA | NA | NA | No |
| ACC-P34 | F/62 | Yes | 4 | Liver | 0.781 | No variants identified | No | NA | 20a | NA | No |
| ACC-P35 | F/58 | Yes | 4 | Liver | 5.758 | No variants identified | No | NA | 75a | NA | No |
| ACC-P36 | F/59 | Yes | 4 | Liver, lung and LN | 2.800 | MEN1 (NM_130799.2) | No | NA | 20a | NA | No |
| Patient-ID . | Sex/age . | Symptoms . | ENSAT tumour stage . | Metastasis at diagnosis . | ccfDNA levels (ng/µL of plasma) . | ccfDNA sequencing - Gene name . | Surgery . | R status . | Ki67 index . | S-GRAS score . | Available Follow-up . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| ACC-P1 | F/55 | Yes | 4 | Liver, lung, LN | 0.195 | CTNNB1(NM_001098209.1) | No | NA | NA | NA | No |
| ACC-P2 | M/83 | Yes | 3 | None | 0.301 | No variants identified | Yes | 0 | 9 | 3 | Long |
| ACC-P3 | M/43 | Yes | 3 | None | Yes | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 0 | 30 | 4 | Long |
| ACC-P4 | M/71 | No | 3 | None | 0.173 | No variants identified | Yes | 0 | 40 | 4 | Short |
| ACC-P5 | F/23 | Yes | 4 | Lung | 1.935 | No variants identified | Yes | 2 | 40 | 8 | Short |
| ACC-P6 | M/36 | Yes | 2 | None | 0.151 | No variants identified | Yes | 0 | 3 | 1 | Long |
| ACC-P7 | F/68 | Yes | 4 | Liver | 2.325 | No variants identified | Yes | 2 | 30 | 9 | No |
| ACC-P8 | F/74 | No | 3 | None | 1.120 | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 1 | 40 | 6 | Short |
| ACC-P9 | M/37 | Yes | 4 | Liver, lung | 0.735 | TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P10 | F/54 | Yes | 4 | Lung | 2.700 | ZNRF3 (NM_001206998.1) | No | NA | NA | NA | No |
| ACC-P11 | M/64 | Yes | 4 | Liver | 4.350 | CTNNB1 (NM_001098209.1) DAXX (NM_001141970.1 RB1 (NM_000321.2) TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P12 | F/73 | Yes | 4 | Liver | 6.000 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P13 | M/57 | Yes | 4 | Liver | 0.401 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P14 | F/36 | Yes | 3 | None | 0.193 | No variants identified | Yes | 1 | 30 | 6 | Short |
| ACC-P15 | F/57 | Yes | 3 | None | 0.890 | No variants identified | Yes | 1 | 10 | 6 | Long |
| ACC-P16 | M/50 | No | 2 | None | 0.062 | NF1 (NM_000267.3)b | Yes | 0 | 17 | 1 | Short |
| ACC-P17 | F/65 | Yes | 3 | None | 0.385 | GNAS (NM_000516.5) | Yes | 1 | 25 | 7 | Short |
| ACC-P18 | F/60 | Yes | 4 | Lung, LN | 5.500 | CTNNB1 (NM_001098209.1) ATM (NM_000051.3)b | No | NA | NA | NA | No |
| ACC-P21 | F/56 | No | 2 | None | 0.251 | No variants identified | Yes | 0 | 30 | 3 | Long |
| ACC-P22 | F/50 | No | 3 | None | 0.247 | No variants identified | Yes | 0 | 18 | 3 | Long |
| ACC-P23 | F/27 | Yes | 3 | None | 0.189 | No variants identified | Yes | 0 | 40 | 4 | No |
| ACC-P24 | M/31 | Yes | 2 | None | 0.163 | No variants identified | Yes | 0 | 12 | 2 | Long |
| ACC-P25 | F/56 | No | 3 | None | 0.454 | No variants identified | Yes | 0 | 5 | 2 | Long |
| ACC-P26 | F/39 | No | 2 | None | 0.185 | CTNNB1 (NM_001098209.1) | Yes | 0 | 40 | 2 | Short |
| ACC-P27 | M/64 | No | 1 | None | 0.114 | No variants identified | Yes | 0 | 23 | 3 | No |
| ACC-P28 | F/35 | Yes | 4 | Lung | 1.473 | TERT (NM_198253.2) (NM_002734.4) | Yes | 2 | 80 | 8 | Short |
| ACC-P29 | F/56 | Yes | 3 | None | 0.302 | No variants identified | Yes | 0 | 12 | 4 | Short |
| ACC-P30 | F/53 | Yes | 2 | None | 0.368 | APC (NM_000038.5) | Yes | 0 | 30 | 4 | Long |
| ACC-P31 | F/51 | Yes | 2 | None | 1.380 | ATM (NM_000051.3)b | Yes | 0 | 22 | 4 | Long |
| ACC-P32 | F/26 | Yes | 4 | Lung | 0.414 | No variants identified | No | NA | 19a | NA | No |
| ACC-P33 | M/48 | Yes | 4 | Liver, lung | 0.846 | No variants identified | No | NA | NA | NA | No |
| ACC-P34 | F/62 | Yes | 4 | Liver | 0.781 | No variants identified | No | NA | 20a | NA | No |
| ACC-P35 | F/58 | Yes | 4 | Liver | 5.758 | No variants identified | No | NA | 75a | NA | No |
| ACC-P36 | F/59 | Yes | 4 | Liver, lung and LN | 2.800 | MEN1 (NM_130799.2) | No | NA | 20a | NA | No |
R status = resection status of primary tumour, S-GRAS score calculated as previously published (Elhassan YS et al., Eur J Endocrinol 2021, DOI: 10.1530/EJE-21-0510). Long follow-up: at least 9 months. Short follow-up: at least 3 months.
Abbreviations: ACC, adrenocortical carcinoma; ccfDNA, circulating cell-free DNA; ENSAT, European Network for the Study of Adrenocortical Tumors; F, female; LN, lymph node; M, male; NA, not applicable.
aki67 index available from adrenal tumour biopsy.
bKnown drug targetable genes according to OncoKb database.
Demographic, clinical, and histopathological characteristics of 34 patients with primary ACC and results of ccfDNA analysis.
| Patient-ID . | Sex/age . | Symptoms . | ENSAT tumour stage . | Metastasis at diagnosis . | ccfDNA levels (ng/µL of plasma) . | ccfDNA sequencing - Gene name . | Surgery . | R status . | Ki67 index . | S-GRAS score . | Available Follow-up . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| ACC-P1 | F/55 | Yes | 4 | Liver, lung, LN | 0.195 | CTNNB1(NM_001098209.1) | No | NA | NA | NA | No |
| ACC-P2 | M/83 | Yes | 3 | None | 0.301 | No variants identified | Yes | 0 | 9 | 3 | Long |
| ACC-P3 | M/43 | Yes | 3 | None | Yes | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 0 | 30 | 4 | Long |
| ACC-P4 | M/71 | No | 3 | None | 0.173 | No variants identified | Yes | 0 | 40 | 4 | Short |
| ACC-P5 | F/23 | Yes | 4 | Lung | 1.935 | No variants identified | Yes | 2 | 40 | 8 | Short |
| ACC-P6 | M/36 | Yes | 2 | None | 0.151 | No variants identified | Yes | 0 | 3 | 1 | Long |
| ACC-P7 | F/68 | Yes | 4 | Liver | 2.325 | No variants identified | Yes | 2 | 30 | 9 | No |
| ACC-P8 | F/74 | No | 3 | None | 1.120 | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 1 | 40 | 6 | Short |
| ACC-P9 | M/37 | Yes | 4 | Liver, lung | 0.735 | TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P10 | F/54 | Yes | 4 | Lung | 2.700 | ZNRF3 (NM_001206998.1) | No | NA | NA | NA | No |
| ACC-P11 | M/64 | Yes | 4 | Liver | 4.350 | CTNNB1 (NM_001098209.1) DAXX (NM_001141970.1 RB1 (NM_000321.2) TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P12 | F/73 | Yes | 4 | Liver | 6.000 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P13 | M/57 | Yes | 4 | Liver | 0.401 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P14 | F/36 | Yes | 3 | None | 0.193 | No variants identified | Yes | 1 | 30 | 6 | Short |
| ACC-P15 | F/57 | Yes | 3 | None | 0.890 | No variants identified | Yes | 1 | 10 | 6 | Long |
| ACC-P16 | M/50 | No | 2 | None | 0.062 | NF1 (NM_000267.3)b | Yes | 0 | 17 | 1 | Short |
| ACC-P17 | F/65 | Yes | 3 | None | 0.385 | GNAS (NM_000516.5) | Yes | 1 | 25 | 7 | Short |
| ACC-P18 | F/60 | Yes | 4 | Lung, LN | 5.500 | CTNNB1 (NM_001098209.1) ATM (NM_000051.3)b | No | NA | NA | NA | No |
| ACC-P21 | F/56 | No | 2 | None | 0.251 | No variants identified | Yes | 0 | 30 | 3 | Long |
| ACC-P22 | F/50 | No | 3 | None | 0.247 | No variants identified | Yes | 0 | 18 | 3 | Long |
| ACC-P23 | F/27 | Yes | 3 | None | 0.189 | No variants identified | Yes | 0 | 40 | 4 | No |
| ACC-P24 | M/31 | Yes | 2 | None | 0.163 | No variants identified | Yes | 0 | 12 | 2 | Long |
| ACC-P25 | F/56 | No | 3 | None | 0.454 | No variants identified | Yes | 0 | 5 | 2 | Long |
| ACC-P26 | F/39 | No | 2 | None | 0.185 | CTNNB1 (NM_001098209.1) | Yes | 0 | 40 | 2 | Short |
| ACC-P27 | M/64 | No | 1 | None | 0.114 | No variants identified | Yes | 0 | 23 | 3 | No |
| ACC-P28 | F/35 | Yes | 4 | Lung | 1.473 | TERT (NM_198253.2) (NM_002734.4) | Yes | 2 | 80 | 8 | Short |
| ACC-P29 | F/56 | Yes | 3 | None | 0.302 | No variants identified | Yes | 0 | 12 | 4 | Short |
| ACC-P30 | F/53 | Yes | 2 | None | 0.368 | APC (NM_000038.5) | Yes | 0 | 30 | 4 | Long |
| ACC-P31 | F/51 | Yes | 2 | None | 1.380 | ATM (NM_000051.3)b | Yes | 0 | 22 | 4 | Long |
| ACC-P32 | F/26 | Yes | 4 | Lung | 0.414 | No variants identified | No | NA | 19a | NA | No |
| ACC-P33 | M/48 | Yes | 4 | Liver, lung | 0.846 | No variants identified | No | NA | NA | NA | No |
| ACC-P34 | F/62 | Yes | 4 | Liver | 0.781 | No variants identified | No | NA | 20a | NA | No |
| ACC-P35 | F/58 | Yes | 4 | Liver | 5.758 | No variants identified | No | NA | 75a | NA | No |
| ACC-P36 | F/59 | Yes | 4 | Liver, lung and LN | 2.800 | MEN1 (NM_130799.2) | No | NA | 20a | NA | No |
| Patient-ID . | Sex/age . | Symptoms . | ENSAT tumour stage . | Metastasis at diagnosis . | ccfDNA levels (ng/µL of plasma) . | ccfDNA sequencing - Gene name . | Surgery . | R status . | Ki67 index . | S-GRAS score . | Available Follow-up . |
|---|---|---|---|---|---|---|---|---|---|---|---|
| ACC-P1 | F/55 | Yes | 4 | Liver, lung, LN | 0.195 | CTNNB1(NM_001098209.1) | No | NA | NA | NA | No |
| ACC-P2 | M/83 | Yes | 3 | None | 0.301 | No variants identified | Yes | 0 | 9 | 3 | Long |
| ACC-P3 | M/43 | Yes | 3 | None | Yes | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 0 | 30 | 4 | Long |
| ACC-P4 | M/71 | No | 3 | None | 0.173 | No variants identified | Yes | 0 | 40 | 4 | Short |
| ACC-P5 | F/23 | Yes | 4 | Lung | 1.935 | No variants identified | Yes | 2 | 40 | 8 | Short |
| ACC-P6 | M/36 | Yes | 2 | None | 0.151 | No variants identified | Yes | 0 | 3 | 1 | Long |
| ACC-P7 | F/68 | Yes | 4 | Liver | 2.325 | No variants identified | Yes | 2 | 30 | 9 | No |
| ACC-P8 | F/74 | No | 3 | None | 1.120 | MEN1 (NM_130799.2) ZNRF3 (NM_001206998.1) | Yes | 1 | 40 | 6 | Short |
| ACC-P9 | M/37 | Yes | 4 | Liver, lung | 0.735 | TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P10 | F/54 | Yes | 4 | Lung | 2.700 | ZNRF3 (NM_001206998.1) | No | NA | NA | NA | No |
| ACC-P11 | M/64 | Yes | 4 | Liver | 4.350 | CTNNB1 (NM_001098209.1) DAXX (NM_001141970.1 RB1 (NM_000321.2) TP53 (NM_000546.5) | No | NA | NA | NA | No |
| ACC-P12 | F/73 | Yes | 4 | Liver | 6.000 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P13 | M/57 | Yes | 4 | Liver | 0.401 | KMT2D (NM_003482.3) | No | NA | NA | NA | No |
| ACC-P14 | F/36 | Yes | 3 | None | 0.193 | No variants identified | Yes | 1 | 30 | 6 | Short |
| ACC-P15 | F/57 | Yes | 3 | None | 0.890 | No variants identified | Yes | 1 | 10 | 6 | Long |
| ACC-P16 | M/50 | No | 2 | None | 0.062 | NF1 (NM_000267.3)b | Yes | 0 | 17 | 1 | Short |
| ACC-P17 | F/65 | Yes | 3 | None | 0.385 | GNAS (NM_000516.5) | Yes | 1 | 25 | 7 | Short |
| ACC-P18 | F/60 | Yes | 4 | Lung, LN | 5.500 | CTNNB1 (NM_001098209.1) ATM (NM_000051.3)b | No | NA | NA | NA | No |
| ACC-P21 | F/56 | No | 2 | None | 0.251 | No variants identified | Yes | 0 | 30 | 3 | Long |
| ACC-P22 | F/50 | No | 3 | None | 0.247 | No variants identified | Yes | 0 | 18 | 3 | Long |
| ACC-P23 | F/27 | Yes | 3 | None | 0.189 | No variants identified | Yes | 0 | 40 | 4 | No |
| ACC-P24 | M/31 | Yes | 2 | None | 0.163 | No variants identified | Yes | 0 | 12 | 2 | Long |
| ACC-P25 | F/56 | No | 3 | None | 0.454 | No variants identified | Yes | 0 | 5 | 2 | Long |
| ACC-P26 | F/39 | No | 2 | None | 0.185 | CTNNB1 (NM_001098209.1) | Yes | 0 | 40 | 2 | Short |
| ACC-P27 | M/64 | No | 1 | None | 0.114 | No variants identified | Yes | 0 | 23 | 3 | No |
| ACC-P28 | F/35 | Yes | 4 | Lung | 1.473 | TERT (NM_198253.2) (NM_002734.4) | Yes | 2 | 80 | 8 | Short |
| ACC-P29 | F/56 | Yes | 3 | None | 0.302 | No variants identified | Yes | 0 | 12 | 4 | Short |
| ACC-P30 | F/53 | Yes | 2 | None | 0.368 | APC (NM_000038.5) | Yes | 0 | 30 | 4 | Long |
| ACC-P31 | F/51 | Yes | 2 | None | 1.380 | ATM (NM_000051.3)b | Yes | 0 | 22 | 4 | Long |
| ACC-P32 | F/26 | Yes | 4 | Lung | 0.414 | No variants identified | No | NA | 19a | NA | No |
| ACC-P33 | M/48 | Yes | 4 | Liver, lung | 0.846 | No variants identified | No | NA | NA | NA | No |
| ACC-P34 | F/62 | Yes | 4 | Liver | 0.781 | No variants identified | No | NA | 20a | NA | No |
| ACC-P35 | F/58 | Yes | 4 | Liver | 5.758 | No variants identified | No | NA | 75a | NA | No |
| ACC-P36 | F/59 | Yes | 4 | Liver, lung and LN | 2.800 | MEN1 (NM_130799.2) | No | NA | 20a | NA | No |
R status = resection status of primary tumour, S-GRAS score calculated as previously published (Elhassan YS et al., Eur J Endocrinol 2021, DOI: 10.1530/EJE-21-0510). Long follow-up: at least 9 months. Short follow-up: at least 3 months.
Abbreviations: ACC, adrenocortical carcinoma; ccfDNA, circulating cell-free DNA; ENSAT, European Network for the Study of Adrenocortical Tumors; F, female; LN, lymph node; M, male; NA, not applicable.
aki67 index available from adrenal tumour biopsy.
bKnown drug targetable genes according to OncoKb database.
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