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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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Extra resources for Advances in Human Genetics 8

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138 Ovarian cysts and fibromas have been described as part of the basal cell nevus syndrome and ovarian carcinomas have been reported in the family cancer syndrome. In addition, familial ovarian carcinoma has been reported numerous times. 175 From an intensive study of six families, Fraumeni et al. 90 concluded that ovarian carcinoma is sometimes dominantly inherited. In some families, it is associated with breast cancer, as was also noted by Lynch et al. 1s8 Familial clusters of urinary tract cancers have been reported, particularly renal carcinoma,l1o but also bladder cancer.

This would argue in favor of the origin of the tumors from diploid totipotential cells or spermatogonial cells. These possibilities would also be more in accord with the origin of these tumors before the onset of meiosis in males at puberty, and with the origin of extragonadal teratomas from cells which have not undergone meiosis. 178 Ovary The most common germ cell tumor of the ovary is the benign teratoma, or dermoid cyst. Teratocarcinoma is more likely in children than adults, whereas the reverse is true for dysgerminoma.

The hyperplasia might, on the other hand, be an expression of the inherited mutation itself and affect most or all of the cells in a tissue. 273 This condition is dominantly inherited. More than one of each type of tumor may be found, and the mean number per gene carrier continues to increase with age such that a mean number of four tumors of each type has been estimated to occur by the age of 50-60 years. 1SS Bilateral tumors are very common in both the thyroid and adrenal. The thyroid tumors secrete both calcitonin and histaminase, the pheochromocytomas secrete primarily epinephrine.

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


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