J Korean Soc Endocrinol.  2002 Aug;17(4):564-571.

A Case of Klinefelter's Syndrome with Rathke's Cleft Cyst

Affiliations
  • 1Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
  • 2Department of Neurosurgery, Yonsei University College of Medicine, Seoul, Korea.

Abstract

Klinefelter's syndrome is one of the most common forms of primary hypogonadism presenting with gynecomastia, azospermia and increased follicle-stimulating hormone. It is well known that this syndrome has an increased incidence of neoplasia, especially breast cancer and extragonadal germ cell tumors. However, it is rarely associated with an intracranial tumor of maldevelopmental origin, especially in the suprasellar area. We report, for the first time, a case of Klinefelter's syndrome, with a Rathke's cleft cyst is the patient was a 32-year-old male who was known to have an incidentaloma form brain computed tomography, which was clinically diagnosed as a suprasellar tumor. After operating, the suprasellar mass was confirmed as a Rathke's cleft cyst, and his hormonal abnormality, an elevated level of follicle-stimulating hormone, was not normalized. Therefore, we performed chromosomal analysis, and diagnosed Klinefelter's syndrome with the XXY karyotype.


MeSH Terms

Adult
Brain
Breast Neoplasms
Follicle Stimulating Hormone
Gynecomastia
Humans
Hypogonadism
Incidence
Karyotype
Klinefelter Syndrome*
Male
Neoplasms, Germ Cell and Embryonal
Follicle Stimulating Hormone
Full Text Links
  • JKSE
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr