Tuberculosis and Respiratory Diseases  1996;43(6):1035-1041

doi:10.4046/trd.1996.43.6.1035

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

Yong Jo KIM 1 ; Gyo Seon KWUN ; Young Wo LEE ; Kyung Tae KIM ; Yeon Hee PARK ; Baek Yeol RYOO ; Tae You KIM ; Young Hyuck IM ; Choon Taek LEE ; Yoon Koo KANG ; Kyung Ja CHO ; Jhin Oh LEE ; Tae Woong KANG

Affiliations

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Keywords

Klinefelter's syndrome; primary mediastinal germ cell tumor

Country

Republic of Korea

Language

Korean

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 reported 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 findings 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 teratoma and endodermal sinus tumor. He was treated with cis-platin containing chemotherapy and followed up in partial remission.