TABLE 2.
Characteristics of cancer-related deaths in the cohort by the presence of genetic abnormalities.
| Genetic abnormalities (N = 7532) |
||||
|---|---|---|---|---|
| Variable | No genetic abnormalities (N = 50,069) |
Down syndrome n = 4949 |
Othera n = 2583 |
p-Valueb |
| Cancer-related deaths | 152 | 42 | 14 | |
| Sex—female | 64 (42.0) | 20 (47.6) | 8 (57.1) | .309 |
| Race | .013 | |||
| White | 52 (34.2) | 12 (28.6) | 2 (14.3) | |
| Black | 10 (6.6) | – | 2 (14.3) | |
| Otherc | 3 (2.0) | 2 (4.8) | – | |
| Unknown | 87 (57.2) | 28 (66.7) | 10 (71.4) | |
| Median age at first intervention, years | 1.31 (0.03–6.27) | 0.63 (0.25–2.34) | 1.91 (0.17–9.67) | .635 |
| Median age at death, years (IQR) | 19.8 (9.8–27.1) | 12.7 (7.5–20.9) | 21.7 (8.7–28.8) | .060 |
| Broad cancer diagnosis type | <.001 | |||
| Solid | 99 (65.1) | 9 (21.4) | 10 (71.4) | |
| Liquid | 37 (24.4) | 32 (76.2) | 3 (21.4) | |
| Unclassifiable | 16 (10.5) | 1 (2.4) | 1 (7.2) | |
| Cancer organ/location (NCI designations) | .002 | |||
| Digestive/gastrointestinal | 31 (20.8) | 1 (2.4) | 1 (7.2) | |
| Endocrine and neuroendocrine | 2 (1.3) | – | 1 (7.2) | |
| Eye | 1 (0.7) | – | – | |
| Genitourinary/gynecologic/breast | 9 (6.0) | – | 1 (7.2) | |
| Head and neck | 2 (1.3) | 1 (1.8) | – | |
| Hematologic/blood | 58 (37.7) | 39 (92.9) | 6 (42.9) | |
| Multiple primary | 4 (2.6) | – | – | |
| Musculoskeletal/skeletal | 7 (4.6) | – | 2 (14.3) | |
| Central/peripheral nervous system | 20 (13.0) | – | 2 (14.3) | |
| Respiratory/thoracic | 4 (2.6) | – | – | |
| Skin | 3 (2.0) | – | – | |
| Unknown | 11 (7.8) | 1 (2.4) | 1 (7.2) | |
| Additional malignancyd | .742 | |||
| Yes | 13 (8.6) | 2 (4.8) | 2 (14.3) | |
| CHD primarydiagnosis | <.001 | |||
| PDA | 14 (9.2) | 3 (7.1) | – | |
| Left to right shunte | 19 (12.5) | 31 (73.8) | 2 (14.3) | |
| LHOL | 21 (13.8) | – | 7 (50.0) | |
| APVR | 6 (4.0) | – | – | |
| RVOTO | 14 (9.2) | 4 (9.5) | 2 (14.3) | |
| TGA physiology | 8 (5.3) | – | – | |
| Truncus arteriosus | 1 (0.7) | – | – | |
| Miscellaneous physiologyf | 30 (19.7) | 3 (7.1) | 2 (14.3) | |
| Single ventricle | 39 (25.7) | 1 (2.4) | – | |
| CHD severity | <.001 | |||
| Mild | 28 (18.4) | 8 (19.1) | 2 (14.3) | |
| Moderate | 34 (22.4) | 29 (69.1) | 6 (42.9) | |
| Severe 2V | 29 (19.4) | 3 (7.1) | 3 (21.4) | |
| Single ventricle | 39 (25.7) | 1 (2.4) | – | |
| Unclassifiable | 22 (14.5) | 1 (2.4) | 3 (21.4) | |
| Cyanotic | .003 | |||
| No | 74 (48.7) | 34 (80.9) | 8 (57.1) | |
| Yes | 66 (43.4) | 8 (19.1) | 3 (21.4) | |
| Unclassifiable | 12 (7.9) | 0 (0.0) | 3 (21.4) | |
Abbreviations: APVR, anomalous pulmonary venous return; CHD, congenital heart defect; LHOL, left heart obstructive lesion; NCI, National Cancer Institute; PDA, patent ductus arteriosus; RVOTO, right ventricular outflow tract obstruction; TGA, transposition of the great arteries.
Includes Marfan, DiGeorge, Noonan, Williams, Turner, and miscellaneous other genetic syndromes and conditions.
p-Values compare overall group with and without genetic abnormalities.
Includes “Asian,” “Native American,” “More than one,” “Pacific Islander,” and “Other.”
Conditions indicated as “secondary malignant neoplasms” on their death record or listed multiple malignancies as contributing causes of death.
Other than PDA.
Miscellaneous lesions not classifiable by a single major pathophysiology.