A Case of Primary Mediastinal Germ Cell Tumor Associated with Klinefelter's Syndrome

Klinefelter 증후군에 병발된 원발성 종격동 생식세포종 1예

  • Kim, Yong-Jo (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Kwun, Gyo-Seon (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Lee, Young Wo (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Kim, Kyung-Tae (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Park, Yeon-Hee (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Ryoo, Baek-Yeol (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Kim, Tae You (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Im, Young-Hyuck (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Lee, Choon-Taek (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Kang, Yoon-Koo (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Cho, Kyung Ja (Department of Pathology, Korea Cancer Center Hospital) ;
  • Lee, Jhin-Oh (Department of Internal Medicine, Korea Cancer Center Hospital) ;
  • Kang, Tae-Woong (Department of Internal Medicine, Korea Cancer Center Hospital)
  • Published : 1996.12.30

Abstract

Klinefelter's syndrome is characterized by small testes, azoospermia, gynecomastia, and elevated levels of plasma gonadotropins in men with two or more X chromosomes. Previous investigators reponed that patients with Klinefelter's syndrome are predisposed to the development of a non-seminomatous germ cell tumor in the mediastinum. It is suggested that this linkage may be due to the hormonal imbalance in Klinefelter's syndrome and consequently, the formation of dysgenetic germ cell and/or abnomal migration of germ cell We report here a case of Klinefelter's syndrome in a 24-years-old man who was presented with anterior mediastinal mass. The clinical and laborarotory fmdings were compatible with Klinefelter's syndrome and he was found to have 47 XXY karyotype. Pathological findings for mediastinal mass revealed mixed germ cell tumor composed of mature cystic teraloma and endodermal sinus rumor. He was treated with cis platin containing chemotherapy and followed up in partial remission.

저자등은 Klinefelter 증후군에 병발된 원발성 종격동 혼합형 생식세포종(mixded germ cell tumor)을 가진 환자 1예를 경험하였기에 이에 문헌고찰과 함께 보고하는 바이다.

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