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Complete Testicular Feminization Syndrome - a Case Report -


JKAPS 2006 Jun;12(1):47-52
Published online June 30, 2006
Copyright © 2006 Korean Association of Pediatric Surgeons.

Ma Hae Cho, M.D., Eun-Sook Nam, M.D.1

Pyungchon Daehang Surgical Clinic, Anyang, Department of Pathology1, Hallym University, Seoul, Korea
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
 Abstract
Testicular feminization syndrome (TFS) is a genetic disorder due to androgen insensitivity of the target organs. The most common clinical presentation of complete TFS is inguinal hernia in the infant or primary amenorrhea in the adolescence. A 7-year old phenotypically female patient was seen with a complaint of a right inguinal mass. Under the diagnosis of right inguinal hernia, high ligation was performed. Six months later, the patient showed a left inguinal mass. On operation, the mass looked like a testis. The external genitalia were normal female, but a uterus and ovary were not identified. Chromosome study showed a 46, XY karyotype and the levels of serum testosterone and dihydrotestosterone were increased after HCG stimulation. The patient was diagnosed as complete TFS and underwent bilateral gonadectomy 6 months later. (J Kor Assoc Pediatr Surg 12(1):47~52), 2006.
Keywords : Testicular feminization syndrome, Androgen insensitivity syndrome, Inguinal hernia

 

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