Table 4.
Mortality from subsequent malignant neoplasms in 1-year survivors of hereditary retinoblastoma, by radiotherapy status*
| Cancer site (ICD-8 code) by radiation dose category | Radiotherapy |
No or uncertain radiotherapy |
||
| O | SMR (95% CI) | O | SMR (95% CI) | |
| Heavily irradiated sites (≥1 Gy) | ||||
| Bone (170)† | 54 | 673 (506 to 879) | 2 | 143 (16 to 519) |
| Connective tissue (171)‡ | 31 | 395 (268 to 560) | 0 | 0 (0 to 234) |
| Brain and other parts of nervous system (191–192) | 10 | 30 (14 to 55) | 0 | 0 (0 to 55) |
| Nasal cavities (160) | 5 | 981 (316 to 2290) | 0 | 0 (0 to 2963) |
| Buccal cavity and pharynx (140–149) | 2 | 44 (4.9 to 158) | 0 | 0 (0 to 282) |
| Moderately irradiated sites (0.4–1.0 Gy) | ||||
| Cutaneous melanoma (172) | 11 | 94 (47 to 168) | 2 | 68 (7.6 to 245) |
| Lung and trachea (162) | 4 | 8.2 (2.2 to 21) | 4 | 24 (6.4 to 61) |
| Breast (174) | 2 | 5.8 (0.7 to 21) | 0 | 0 (0 to 32) |
| Leukemia (204–207) | 1 | 2.0 (0.03 to 11) | 0 | 0 (0 to 42) |
| Lightly irradiated sites (<0.4 Gy) | ||||
| Corpus uteri (182)§ | 4 | 164 (44 to 421) | 1 | 122 (1.6 to 676) |
| Large intestine (153) | 1 | 6.2 (0.08 to 35) | 0 | 0 (0 to 75) |
| Bladder (188) | 1 | 53 (0.7 to 297) | 0 | 0 (0 to 602) |
| All malignant neoplasms other than retinoblastoma‖ | 140 | 41 (35 to 49) | 11 | 12 (6.1 to 22) |
| Absolute excess risk per 10 000 person-years | 56.6 | 24.6 | ||
Number of persons followed up = 956 for radiotherapy and 136 for no or uncertain radiotherapy; person-years of follow-up = 24 146 for radiotherapy and 4104 for no or uncertain radiotherapy. List of malignant neoplasms under each radiation dose category 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.
Locations of bone cancers in irradiated survivors include 17 of the skull and face (ICD-8 170.0), four of the long bones of lower limb (ICD-8 170.7), and 33 of unspecified site (ICD-8 170.9). Locations of bone cancers in nonirradiated survivors include one of the ribs (ICD-8 170.3) and one of unspecified site (ICD-8 170.9).
Locations of connective tissue cancers in irradiated survivors include five of the head, face, and neck (ICD-8 171.0), and 26 of unspecified site (ICD-8 171.9).
Includes three leiomyosarcomas, one carcinoma, and one Müllerian mixed tumor.
Cancer sites not listed for irradiated survivors include two each of retroperitoneal tissue (ICD-8 158.0), and nonmelanoma skin cancer (ICD-8 173.4, 173.9); one each of ovary (ICD-8 183.0), kidney (ICD-8 189.0), pineal gland (ICD-8 194.4), and abdomen, ill-defined (ICD-8 195.0), and six of cancer, not otherwise specified (ICD-8 199). Cancer sites not listed for nonirradiated survivors include one each of lymphoid tissue (ICD-8 202.2), and cancer, not otherwise specified (ICD-8 199).