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Subsequent Neoplasms

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    Subsequent Neoplasms and Genetic Susceptibility

    Literature clearly supports the role of chemotherapy and radiation in the development of SNs. However, interindividual variability exists, suggesting that genetic variation has a role in susceptibility to genotoxic exposures, or that genetic susceptibility syndrome confers an increased risk of cancer, such as Li-Fraumeni syndrome. Previous studies have demonstrated that childhood cancer survivors with either a family history of cancer, but more so, presence of Li-Fraumeni syndrome, carry an increased risk of developing an SN.[48,49] The risk of SNs could potentially be modified by mutations in high-penetrance genes that lead to these serious genetic diseases (e.g., Li-Fraumeni syndrome).[49] However, the attributable risk is expected to be very small because of the extremely low prevalence of mutations in high-penetrance genes. Table 1 below summarizes the spectrum of neoplasms, affected genes, and Mendelian mode of inheritance of selected syndromes of inherited cancer predisposition.

    Table 1. Selected Syndromes of Inherited Cancer Predispositiona

    SyndromeMajor Tumor TypesAffected GeneMode of Inheritance
    WAGR = Wilms tumor, aniridia, genitourinary anomalies, mental retardation.
    a Adapted from Strahm et al.[50]
    Adenomatous polyposis of the colonColon, hepatoblastoma, intestinal cancers, stomach, thyroid cancerAPCDominant
    Ataxia-telangiectasiaLeukemia, lymphomaATMRecessive
    Beckwith-Wiedemann syndromeAdrenal carcinoma, hepatoblastoma, rhabdomyosarcoma, Wilms tumorCDKN1C/NSD1Dominant
    Bloom syndromeLeukemia, lymphoma, skin cancerBLMRecessive
    Fanconi anemiaGynecological tumors, leukemia, squamous cell carcinomaFANCA, FANCB, FANCC, FANCD2, FANCE, FANCF, FANCGRecessive
    Juvenile polyposis syndromeGastrointestinal tumorsSMAD4/DPC4Dominant
    Li-Fraumeni syndromeAdrenocortical carcinoma, brain tumor, breast carcinoma, leukemia, osteosarcoma, soft tissue sarcomaTP53Dominant
    Multiple endocrine neoplasia 1Pancreatic islet cell tumor, parathyroid adenoma, pituitary adenomaMEN1Dominant
    Multiple endocrine neoplasia 2Medullary thyroid carcinoma, pheochromocytomaRETDominant
    Neurofibromatosis type 1Neurofibroma, optic pathway glioma, peripheral nerve sheath tumorNF1Dominant
    Neurofibromatosis type 2Vestibular schwannomaNF2Dominant
    Nevoid basal cell carcinoma syndromeBasal cell carcinoma, medulloblastomaPTCHDominant
    Peutz-Jeghers syndromeIntestinal cancers, ovarian carcinoma, pancreatic carcinomaSTK11Dominant
    RetinoblastomaOsteosarcoma, retinoblastomaRB1Dominant
    Tuberous sclerosisHamartoma, renal angiomyolipoma, renal cell carcinomaTSC1/TSC2Dominant
    von Hippel-Lindau syndromeHemangioblastoma, pheochromocytoma, renal cell carcinoma, retinal and central nervous tumorsVHLDominant
    WAGR syndromeGonadoblastoma, Wilms tumorWT1Dominant
    Wilms tumor syndromeWilms tumorWT1Dominant
    Xeroderma pigmentosumLeukemia, melanomaXPA, XPB, XPC, XPD, XPE, XPF, XPG, POLHRecessive
    1|2|3|4|5|6

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