<abstract xmlns="http://www.w3.org/1999/xhtml"> Background: Klinefelter syndrome may present as precocious puberty, which can be either central precocious puberty or peripheral precocious puberty, caused by an extragonadal germ cell tumor.Objective: Report two cases of Klinefelter syndrome that presented with precocious puberty due to a β-hCG producing mediastinal tumor.Method: Review of the clinical history, physical examination, and laboratory investigations.Results: Pseudo-precocity developed some years before diagnosis of -hCG producing tumor. The patients did not have typical physical features of this syndrome. The testes were small and had loose consistency.Conclusion: Klinefelter syndrome must be excluded in all boys presenting with precocious puberty due to a β-hCG producing tumor. Conversely, patients with Klinefelter syndrome should be regularly checked for β-hCG and α-fetoprotein levels. In those cases, the patients can be diagnosed and treated early. With the early treatment, they will be able to attain normal adult height and have fewer complications from the tumor.</abstract>

Background: Klinefelter syndrome may present as precocious puberty, which can be either central precocious puberty or peripheral precocious puberty, caused by an extragonadal germ cell tumor.Objective: Report two cases of Klinefelter syndrome that presented with precocious puberty due to a β-hCG producing mediastinal tumor.Method: Review of the clinical history, physical examination, and laboratory investigations.Results: Pseudo-precocity developed some years before diagnosis of -hCG producing tumor. The patients did not have typical physical features of this syndrome. The testes were small and had loose consistency.Conclusion: Klinefelter syndrome must be excluded in all boys presenting with precocious puberty due to a β-hCG producing tumor. Conversely, patients with Klinefelter syndrome should be regularly checked for β-hCG and α-fetoprotein levels. In those cases, the patients can be diagnosed and treated early. With the early treatment, they will be able to attain normal adult height and have fewer complications from the tumor.

Two cases of Klinefelter syndrome with precocious puberty due to mediastinal germ cell tumor

MD, PhD, Division of Endocrinology, Department of Pediatrics, Chulalongkorn University, Bangkok 10330,

Division of Endocrinology, Department of Pediatrics, Faculty of Medicine, Chulalongkorn University, Bangkok 10330,

Asian Biomedicine

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Background: Klinefelter syndrome may present as precocious puberty, which can be either central precocious puberty or peripheral precocious puberty, caused by...

Two cases of Klinefelter syndrome with precocious puberty due to mediastinal germ cell tumor

Article Category: Clinical report

Published Online: Feb 04, 2017

Page range: 417 - 421

DOI: https://doi.org/10.5372/1905-7415.0503.055

Keywords
Klinefelter syndrome, mediastinal germ cell tumor, precocious puberty

© 2017

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Two cases of Klinefelter syndrome with precocious puberty due to mediastinal germ cell tumor

Article Category: Clinical report

Published Online: Feb 04, 2017

Page range: 417 - 421

DOI: https://doi.org/10.5372/1905-7415.0503.055

KeywordsKlinefelter syndrome, mediastinal germ cell tumor, precocious puberty

© 2017

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 License.

Keywords
Klinefelter syndrome, mediastinal germ cell tumor, precocious puberty