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By Alfred G. Knudson Jr. (auth.), Harry Harris, Kurt Hirschhorn (eds.)

ISBN-10: 1461582679

ISBN-13: 9781461582670

ISBN-10: 1461582695

ISBN-13: 9781461582694

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Knudson, Jr. the second meiotic division (or its equivalent, fusion of its products), as has been so beautifully demonstrated for ovarian teratomas by Linder et al. 177 If this be the case, then some testicular tumors should originate from an XX cell and some from a YY cell. Unfortunately, this question has not been settled, although tumors have been found both with and without a Y chromosome. 20 1 On the other hand, Atkin 16 reported that sex chromatin and Y bodies were both consistently present in several testicular teratomas.

148 Other tumors, especially medulloblastoma and ovarian fibroma, also occur in the syndrome. It is interesting that more differentiated gliomas are not found. Patients with the syndrome and medulloblastoma who have been treated with radiation to the cranium and spinal axis develop large numbers of basal cell carcinomas in the field of irradiation within 6 months to 3 years of the treatment. 279 Such an effect has not been observed among similarly treated cases without the syndrome. The greatly enhanced oncogenic effect of radiation and the marked shortening of the usual latent period have been interpreted by Strong279 to favor the idea that the radiation has produced a second mutation in cells that already carried a first mutation and that doubly mutated cells develop into tumors.

Each occurs almost exclusively in children and each is thought to arise from transient embryonic stem cells that disappear by differentiation. Each has an incidence of 5-10 per 100,000 births. Retinoblastoma has been noted already as a common accompaniment of interstitial deletion of chromosome 13; such a deletion is not found in the usual dominant form. 163 Although the cases of 13-deletion are few, the distribution of patients' ages at diagnosis and the frequency of Chapter 1: Genetics and Etiology of Human Cancer 35 bilateral tumors are both almost identical to these features in hereditary cases without deletion.

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Advances in Human Genetics 8 by Alfred G. Knudson Jr. (auth.), Harry Harris, Kurt Hirschhorn (eds.)


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