Table 2.
Causes of death other than retinoblastoma in 1-year survivors of retinoblastoma, by hereditary status*
| Cause of death† (ICD-8 code) | Hereditary |
Nonhereditary |
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| O | SMR (95% CI) | AER | O | SMR (95% CI) | AER | |
| Malignant and benign neoplasms other than retinoblastoma | 160 | 37 (31 to 43) | 55.1 | 12 | 2.5 (1.3 to 4.3) | 3.3 |
| Malignant neoplasms other than retinoblastoma‡ | 151 | 35 (30 to 41) | 51.9 | 12 | 2.5 (1.3 to 4.4) | 3.3 |
| Bone (170) | 56 | 595 (449 to 773) | 19.8 | 0 | 0 (0 to 49) | 0.0 |
| Connective tissue (171) | 31 | 329 (223 to 467) | 10.9 | 0 | 0 (0 to 44) | 0.0 |
| Melanoma (172) | 13 | 89 (47 to 151) | 4.5 | 0 | 0 (0 to 25) | −0.1 |
| Brain and other parts of nervous system (191–192) | 10 | 25 (12 to 46) | 3.4 | 2 | 5.9 (0.7 to 21) | 0.8 |
| Brain (191) | 6 | 18 (6.5 to 39) | 2.0 | 2 | 6.7 (0.8 to 24) | 0.8 |
| Other parts of nervous system (192)§ | 4 | 61 (16 to 155) | 1.4 | 0 | 0 (0 to 80) | 0.0 |
| Lung and trachea (162) | 8 | 12 (5.3 to 24) | 2.6 | 0 | 0 (0 to 3.8) | −0.5 |
| Corpus uteri (182)‖ | 5 | 154 (50 to 359) | 1.8 | 1 | 24 (0.3 to 134) | 0.4 |
| Nasal cavities (160) | 5 | 790 (254 to 1843) | 1.8 | 0 | 0 (0 to 570) | 0.0 |
| Breast (174) | 2 | 4.4 (0.5 to 16) | 0.5 | 3 | 5.7 (1.2 to 17) | 1.1 |
| Buccal cavity and pharynx (140–149)¶ | 2 | 34 (3.8 to 123) | 0.7 | 0 | 0 (0 to 51) | −0.03 |
| Leukemia (204–207)# | 1 | 1.7 (0.02 to 9.5) | 0.1 | 1 | 2.2 (0.03 to 12) | 0.2 |
| Thyroid (193) | 0 | 0 (0 to 436) | 0.0 | 1 | 102 (1.3 to 568) | 0.5 |
| Bladder (188) | 1 | 40 (0.5 to 224) | 0.3 | 0 | 0 (0 to 90) | 0.0 |
| Benign neoplasms (210–239)** | 9 | 72 (33 to 137) | 3.1 | 0 | 0 (0 to 33) | −0.1 |
| Other known causes of death | 19 | 0.9 (0.5 to 1.4) | −0.9 | 20 | 1.0 (0.6 to 1.6) | 0.2 |
| Infections (000–139) | 1 | 0.7 (0.01 to 3.9) | −0.1 | 2 | 1.7 (0.2 to 6.0) | 0.4 |
| Endocrine and metabolic diseases (240–279) | 1 | 1.5 (0.02 to 8.4) | 0.1 | 1 | 1.5 (0.02 to 8.3) | 0.2 |
| Mental disorders (290–315) | 0 | 0 (0 to 11) | −0.1 | 3 | 9.7 (2.0 to 28) | 1.2 |
| Neurological diseases (320–389) | 2 | 2.9 (0.3 to 10) | 0.5 | 0 | 0 (0 to 6.0) | −0.3 |
| Circulatory diseases (390–458) | 4 | 1.3 (0.3 to 3.2) | 0.3 | 3 | 0.7 (0.2 to 2.2) | −0.5 |
| Arteriosclerotic heart disease (410–414) | 3 | 2.0 (0.4 to 5.7) | 0.5 | 1 | 0.5 (0.01 to 2.6) | −0.5 |
| Cerebrovascular accidents (430–438) | 1 | 2.1 (0.03 to 11) | 0.2 | 0 | 0 (0 to 6.5) | −0.3 |
| Respiratory diseases (460–519) | 2 | 2.3 (0.3 to 8.2) | 0.4 | 0 | 0 (0 to 3.7) | −0.5 |
| Digestive system diseases (520–579) | 3 | 3.3 (0.7 to 9.5) | 0.7 | 2 | 2.1 (0.2 to 7.6) | 0.5 |
| External causes (800–998) | 6 | 0.5 (0.2 to 1.0) | 2.4 | 9 | 0.9 (0.4 to 1.7) | 0.5 |
| Ill-defined conditions (796) | 6 | 13 (4.7 to 28) | 2.0 | 4 | 9.9 (2.7 to 25) | 1.7 |
Number of persons followed up = 1092 for hereditary and 762 for nonhereditary; person-years of follow-up = 28 250 for hereditary and 21 674 for nonhereditary. List of malignant neoplasms is based on observed numbers of deaths in the combined cohort (in descending order). ICD-8 = International Classification of Diseases, 8th revision; O = observed number of deaths; SMR = standardized mortality ratio; CI = confidence interval; AER = absolute excess risk of death per 10 000 person-years.
Death certificate was not available for 13 hereditary retinoblastoma survivors and three nonhereditary retinoblastoma survivors.
Cancer sites not listed for hereditary retinoblastoma survivors include two of retroperitoneal tissue (ICD-8 158.0); one each of colon (ICD-8 153.0), nonmelanoma skin cancer of the scalp and neck (ICD-8 173.4), nonmelanoma skin cancer, site unspecified (ICD-8 173.9), ovary (ICD-8 183.0), kidney (ICD-8 189.0), pineal gland (ICD-8 194.4), lymphoid tissue (ICD-8 202.2), and abdomen, ill-defined (ICD-8 195.0), and seven of cancer, not otherwise specified (ICD-8 199). Cancer sites not listed for nonhereditary retinoblastoma survivors include four of cancer, not otherwise specified (ICD-8 199).
Includes four neuroblastoma (ICD-8 192.5).
Includes three leiomyosarcomas, one carcinoma, and one Müllerian mixed tumor among hereditary retinoblastoma survivors. No additional information was available for the nonhereditary retinoblastoma survivor who died of cancer of the corpus uteri.
Both nasopharynx (ICD-8 147).
Includes one acute lymphoid leukemia (ICD-8 204.0) in a hereditary retinoblastoma survivor and one acute myeloid leukemia (ICD-8 205.0) in a nonhereditary retinoblastoma survivor.
Benign tumors included one meningioma of the spine (ICD-8 225.4), one benign neoplasm of the pituitary gland and craniopharyngeal duct (ICD-8 226.2), and seven brain neoplasms with unspecified nature (ICD-8 238.1).