Table 2

Numbers of cancer cases, by tumor type and ages at diagnosis, in p53 carriers and their first-degree relatives, after excluding the 6 LFS-associated neoplasmsa

Primary sitep53 mutation carriersbFirst-degree relativesbUnited States populationc
No. of casesMedian age at diagnosis (range) (yrs)No. of casesMedian age at diagnosis (range) (yrs)Median age at diagnosis (yrs)
Lung1150 (18–63)1843 (31–75)68
Stomach835 (20–60)1537 (29–72)71
Ovary842 (21–56)831 (16–40)63
Colon/rectum733 (9–64)1245 (25–65)72
Lymphomas425 (16–37)1132 (21–51)62
Melanoma335 (16–35)345 (44–53)54
Endometrium250 (48–51)13565
Thyroid237 (31–44)344 (24–51)44
Pancreas149243 (36–49)71
Prostate158650 (44–56)73
Cervix130333 (33–62)50
Otherd1227
All types60109
  • a LFS-associated cancers: breast and adrenocortical carcinomas, sarcomas of bone and soft tissues, brain tumors, and acute leukemias.

  • b Combined data from 45 DF/NCI families and 140 additional kindreds in the literature.

  • c SEER Cancer Incidence Public-Use Database, 1973–1995.

  • d Other cancers include: carcinomas of the bladder (3), kidney (2), esophagus (3), and unknown primary site (2); hepatoblastoma (2); neuroblastoma (2); mesothelioma (2); and 23 other neoplasms, including rare carcinomas of the gall bladder, ampulla of Vater, ureter, and testis, and Wilms’ tumor (1 each).