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KMID : 0387820190260020105
Clinical Pediatric Hematology-Oncology
2019 Volume.26 No. 2 p.105 ~ p.109
Embryonal Carcinoma of the Pineal Gland Developed in an Adolescent Boy with Klinefelter Syndrome
Choi Hyo-Jin

Kim Eun-Ah
Lee Jae-Min
Jang Kyung-Mi
Choi Joon-Hyuk
Abstract
Klinefelter syndrome (KS) is characterized by small testes, gynecomastia, tall stature, and hypergonadotropic hypogonadism. This condition is associated with extra X chromosomes. It is well known that these aneuploidies predispose individuals to the development of several cancers. Moreover, there are many case reports that show KS patients to have a higher relative risk for the development of malignancy. However, incracranial germ cell tumor (ICGCT) associated with KS is very uncommon. Herein, we report delayed diagnosis of KS in a 15-year-old boy with ICGCT, embryonal carcinoma of the pineal gland, after multimodality treatment in Korea.
KEYWORD
Klinefelter syndrome, Aneuploidy, Intracranial germ cell tumor
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