Genetic Factors in Solid Tumors of Childhood

  • M. Ponz de Leon
Part of the Recent Results in Cancer Research book series (RECENTCANCER, volume 136)


Childhood cancer is relatively rare in all Western and developed countries. In the United States, approximately 4000 new cases of malignant solid tumors are diagnosed each year [1], while data from cancer registries in Europe indicate that the fraction of all pediatric malignancies (i.e., diagnosed between 0 and 14 years of age) is in the order of 0.8% of all registered tumors [2]. Despite the relative rarity, there are several reasons for interest in these tumors. First, for many cancers of childhood a genetic nature has been suspected, and then documented, by observing the striking familial aggregation and vertical transmission of uncommon tumors; retinoblastoma (Rb) , Wilms' tumor (WT) , and Li-Fraumeni syndrome are examples of well defined hereditary neoplastic diseases of infancy. Second, some of these neoplasms (Rb and WT, in particular) have become a paradigm for interpreting tumor development as being due to the sequential inactivation of the two alleles in a tumor suppressor gene (see the previous chapter) . This model , initially suggested for Rb [3], is applicable to many other clinical conditions which are at present under active investigation (adenomatosis coli, hereditary bre ast and colonic cancer). Third, the distribution of childhood cancer is rather peculiar and markedly different from that of adult malignant tumors, as shown in Fig. 1. In fact, tumors of the Iymphohematopoietic system represent 50% of all malignancies developed before the age of 15, brain tumors 22%, and sarcoma 11%; all together, carcinomas do not reach 6% of tot al pediatric tumors. Cancer distribution is entirely different in adults , where carcinomas represent the large majority (89% of total), and altogether the most frequent neoplasms of childhood do not reach 12% of all registered tumors. The reasons for this striking difference in cancer distribution are unclear, though rather intuitively one may suspect that genetic factors affect pediatric tumors more than the adult types, which in turn should be more closely dependent on the cumulative effect of environmental factors.


Childhood Cancer Mesoblastic Nephroma Hereditary Case Adrenal Medullary Hormone Painless Abdominal Mass 
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Copyright information

© Springer-Verlag Berlin Heidelberg 1994

Authors and Affiliations

  • M. Ponz de Leon
    • 1
  1. 1.Istituto di Patologia Medica PoliclinicoUniversità degli Studi di ModenaModenaItaly

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