Table 4.
Characteristic | All patients | Pathogenic germline variant | No pathogenic germline variant |
---|---|---|---|
Total | 450 | 44 | 94 |
Age at onset, No. (%), y | |||
Median (range) | 46 (15-50) | 46 (24-50) | 45(25-50) |
≤30 | 9 (2.0) | 3 (6.8) | 2 (2.1) |
>30 to ≤40 | 56 (12.4) | 10 (22.7) | 18 (19.1) |
>40 to <50 | 385 (85.6) | 31 (70.5) | 74 (78.7) |
Personal history of a previous cancer, No. (%) | 23 (5.1)a | 6 (13.6) | 9 (9.6) |
Family history of cancer in first- or second-degree family member, No. (%) | 330 (73.3)b | 37 (84.1) | 71 (75.5) |
Family history of cancer in first-degree family member, No. (%) | 203 (45.1) | 28 (63.6) | 42 (44.7) |
Family history of cancer in second-degree family member, No. (%) | 244 (54.2) | 26 (59.1) | 62 (66.0) |
Clinical stage at diagnosis, No. (%) | |||
Resectable | 105 (23.3) | 15 (34.1) | 30 (31.9) |
Locally advanced | 121 (26.9) | 10 (22.7) | 19 (20.2) |
Metastatic | 218 (48.4) | 19 (43.2) | 45 (47.9) |
12-month OS in patients with stage IV disease at diagnosis (95% CI) | 43.6 (36.8 to 50.3) | 72.2 (45.6 to 87.4) | 54.6 (38.9 to 67.9) |
Breast cancer (n = 8), melanoma (n = 4), lymphoma (n = 4), sarcoma (n = 3), pheochromocytoma (n = 2), bladder cancer (n = 1), germ cell tumor (n = 1), thyroid cancer (n = 1), and vulvar cancer (n = 1). CI = confidence interval; OS = overall survival.
56 patients with a family history of pancreas cancer, 13 with bladder cancer, 131 with breast cancer, 82 with colorectal cancer, 70 with lung cancer, 11 with melanoma, 22 with ovarian cancer, 52 with prostate cancer, and 155 with other cancers.