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

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

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Fibromas of the ovary are also a feature of the syndrome. However, Strong279 observed fibrosarcomas arising from fibromas of the ovary in her patient, probably as a result of the previous radiation to the spinal axis. Another case of fibrosarcoma following radiation had been reported previously. Retinoblastoma Retinoblastoma is often considered as a classical example of a dominantly inherited tumor. As is now well known, this is true for only a fraction of retinoblastoma; over half of the cases are not associated with a dominant mutation, as will be discussed below.

The prospect that this relationship may be studied even in human cells is bright in view of the demonstration that the SV40 genome is integrated into human chromosome 7 in transformed, oncogenic fibroblasts. 54 Although Burkitt's lymphoma is not of proven viral origin, it is interesting that chromosome 14 is abnormal iri some cases/96 just as it is in other lymphomas,94 even when those cases occur in patients with ataxia telangiectasia. 122,202 It will be of great interest to discover whether chromosome 14 is an integration site for Epstein-Barr virus,.

41 The latter tumor is also seen as a part of another dominantly inherited condition, Gardner's syndrome. 187 Brain Tumors Two dominantly inherited conditions already discussed are predisposing to brain tumors. In neurofibromatosis both meningioma and glioma are found excessively, while in the basal cell nevus syndrome there is a specificity for medulloblastoma. 183 Brain tumors, often lymphoreticular neoplasms, are also a feature of the immunodeficiency diseases, particularly the Wiskott-Aldrich syndrome.

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