By Alfred G. Knudson Jr. (auth.), Harry Harris, Kurt Hirschhorn (eds.)
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Additional info for Advances in Human Genetics 8
66 In this pedigree, several 32 Alfred G. Knudson, Jr. persons had other forms of cancer as well. Neurofibrosarcoma is often associated with neurofibromatosis, and in some instances so is fibrosarcoma. 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.
The daughter's tumor was bilateral. A paternal aunt had previously died with ovarian cancer, but the type was unknown. Another report203 concerns undifferentiated carcinoma, possibly related to embryonal carcinoma in the male, in three teenage sisters. No Chapter 1: Genetics and Etiology of Human Cancer 39 families have been reported in which teratomas have been found with dysgerminoma. Finally, there are two reports of females affected with ataxia telangiectasia and ovarian tumors, in one case bilateral dysgerminoma,64 in the other dysgerminoma and gonadoblastoma.
1s5 Ganglioneuromas also occur at increased frequency in the syndrome, and neuroblastoma has been recorded toO. 26,153,155,312 This conjunction of all three tumors in one hereditary condition is unique and suggests a different pathogenic mechanism. The relative probability of occurrence of each in neurofibromatosis may be similar to that in the population generally, there being a proportionate increase in the probability of each. 154 Basal Cell Nevus Syndrome Basal cell nevus syndrome, a dominantly inherited condition, is characterized by many stigmatizing features, including cysts of the jaw, 22 Alfred G.