Table 1

Tumor spectrum in p53 mutation carriers and their first-degree relatives within 45 affected DF/NCI kindreds and 140 families in the literature

Tumor typesNo. of cancers
p53 carriersFirst-degree relativesaTotal
DF/NCI casesLiterature casesBoth seriesDF/NCI casesLiterature casesBoth series
Component tumors of LFS6731538284103187569
Breast carcinoma2094114304575189
Soft tissue sarcoma187896151328124
Brain tumors86270232245115
Bone sarcoma14496310162689
Adrenocortical carcinoma3242723532
Leukemia481244820
Other tumors852604069109169
Total75367442124172296738
  • a Relatives with nearly 50% likelihood of a germ-line p53 mutation are parents, siblings, and offspring of carriers.