Patient no. | Sex | Mutation | Age at diagnosis (y) | Location of primary tumor | Biochemical phenotype | Time to metastases* (y) | Locations of metastases | Previous treatment |
---|---|---|---|---|---|---|---|---|
1 | M | SDHB | 27 | L extraadrenal abdominal | None | 0 | Abdominal LN, mediastinum, bone | Primary tumor resection; CVD |
2 | F | SDHB | 51 | R paraaortic abdominal | NE | — | — | MIBG |
3 | F | SDHB | 23 | Paraaortic abdominal | NE | 0.3 | Abdominal LN, bone | Primary tumor resection; CVD |
4 | M | SDHD | 22 | R adrenal | NE | 10.3 | Abdominal LN, liver, mediastinum, bone | Primary tumor resection; MIBG |
5 | F | Pending | 53 | R adrenal | NE | — | — | — |
6 | M | SDHB | 10 | R paraadrenal | NE + DA | 8.4 | Abdominal LN, lung, liver, bone, neck | MIBG |
7 | M | Pending | 39 | R adrenal | NE + DA | 14.7 | Abdominal LN, mediastinum, bone | Primary tumor resection |
8 | F | RET | 33 | R + L adrenal | NE + E | 6.2 | Abdominal LN, liver, bone | Primary tumor resection; MIBG |
9 | F | RET | 33 | R + L adrenal | E | — | — | Primary tumor resection |
10 | M | SDHB | 40 | L paraaortic | NE | 3.9 | Liver | Primary tumor resection |
11 | M | Pending | 40 | R adrenal | NE + DA | 5.4 | Lung, bone | Primary tumor resection; CVD; MIBG |
↵* Time between diagnosis of primary tumor and metastatic disease.
SDHB/D = succinate dehydrogenase subunit B/D; LN = lymph nodes; CVD = cyclophosphamide/vincristine/dacarbazine; NE = norepinephrine; MIBG = 131I-methyliodobenzylguanidine; DA = dopamine; RET = rearranged during transfection; E = epinephrine.