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An ovarian dysgerminoma in Down syndrome. Hypothesis about the association
  1. D. Satgé*,
  2. L. Honoré,
  3. A. J. Sasco,
  4. M. Vekemans§,
  5. A. Chompret and
  1. * Laboratory of Pathology, Centre Hospitalier, Tulle Cedex, France
  2. Laboratory Medicine, Cross Cancer Institute, Edmonton, Alberta, Canada
  3. International Agency for Research on Cancer, Unit of Epidemiology for Cancer Prevention and Institut National de la Santé et de la Recherche Médicale, Lyon Cedex, France
  4. § Laboratory of Cytogenetics, Hôpital Necker, Paris Cedex, France
  5. Department of Genetic Oncology, Institut Gustave Roussy, Villejuif Cedex, France
  6. Centre Médical Jérôme Lejeune, Paris, France
  1. Address correspondence and reprint requests to: Daniel Satgé, Laboratoire d'Anatomie Pathologique, Centre Hospitalier, 19 000 Tulle, France. Email: daniel.satge{at}


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.

  • Down syndrome
  • dysgerminoma
  • germ cell tumor
  • ovarian neoplasm
  • trisomy 21

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