Surveillance of the gonads of male patients with DS is recommended. A better understanding of the factors involved could also help to identify risk factors in the general population.
Cerebral tumors in patients with DS have a specific distribution and may behave differently compared with the general population. These features may be related to the gene dosage effect of oncogenes, antioncogenes, and genes involved in cerebral development due to the supernumerary chromosome 21.
An 11-year-old girl with Down syndrome (DS) was operated for a stage I right ovary dysgerminoma. She is in good health 33 years later. Some data in the literature suggest that ovarian cancers could be slightly overrepresented in DS. Despite the rarity of ovarian dysgerminoma, our case is the fifth reported in DS. This case is the second one associated with a family history suggesting the possibility of a familial predisposition to cancer. A hypothesis explaining the development of dysgerminoma in DS is proposed.
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