Dergiler / Turkish Journal of Pediatrics / 2019 / Cilt: 61 - Sayı: 3

A synchronous occurrence of bifocal intracranial germinoma and bilateral testicular epidermoid cyst in an adolescent patient with Klinefelter’s syndrome

Sayfa
456–459
DOI
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Özet

Klinefelter syndrome (KS) is characterized by an additional X chromosome inmales leading to a karyotype of 47,XXY. It is associated with an increased riskof certain malignancies, including leukemia, breast cancer and extragonadalgerm cell tumor such as mediastinal germ cell tumors and rarely intracranialgerm cell tumors. It is possible that the increased risk of developing certaincancers can be attributed to a direct effect of the chromosomal abnormality orthe combined action of the abnormal chromosomes and hormonal imbalances.Here we describe a synchronous occurrence of bifocal intracranial germinomaand bilateral testicular epidermoid cyst in an adolescent patient withKlinefelter’s syndrome. The synchronous occurrence of the dual tumors inthis patient with Klinefelter’s syndrome might be resulted from the migrationdefect during embriyogenesis due to underlying genetic disease or it is acoincidental condition, yet there has been no case reported in the literature,so far.