Literature DB >> 24333107

Complete androgen insensitivity syndrome with a Sertoli-Leydig cell tumor.

Houda Fagouri1, Driss Rahali Moussaoui2, Jaoud Kouach2, Abdelah Babahabib2, Mohamed Oukabli3, Ahmed Ameur4, Abderrahmane Albouzidi3, Mohamed Dehayni2.   

Abstract

BACKGROUND: The complete androgen insensitivity (testicular feminization) syndrome was described in phenotypic females with 46XY karyotype, presenting with primary amenorrhea, adequate breast development, and absent or sparse pubic or axillary hair. Gonads consist usually of seminiferous tubules without spermatogenesis. CASE: We report the case of a 15-year-old girl with testicular feminization since age 4. She was admitted to surgically remove the testes from the inguinal canal. Microscopic examination of the left testis revealed a Sertoli-Leydig cell tumor and the draft of fallopian tube with adjacent seminiferous tubules. SUMMARY AND
CONCLUSION: These patients have a 5%-10% risk of developing germ cell tumors. Sertoli-Leydig cell tumors are uncommon neoplasms, usually benign in testicular feminization syndrome.
Copyright © 2014 North American Society for Pediatric and Adolescent Gynecology. Published by Elsevier Inc. All rights reserved.

Entities:  

Keywords:  Androgen insensitivity syndrome; Orchidectomy; Sertoli-Leydig cell tumors; Testicular feminization

Mesh:

Year:  2013        PMID: 24333107     DOI: 10.1016/j.jpag.2013.08.007

Source DB:  PubMed          Journal:  J Pediatr Adolesc Gynecol        ISSN: 1083-3188            Impact factor:   1.814


  4 in total

1.  Physical assessment and reference growth curves for children with 46, XY disorders of sex development.

Authors:  Di Wu; Hui Chen; Chunxiu Gong
Journal:  Pediatr Investig       Date:  2017-12-27

2.  Response to: Comment on "Complete Androgen Insensitivity Syndrome: Optimizing Diagnosis and Management".

Authors:  Antonio Simone Laganà; Alfonsa Pizzo
Journal:  Case Rep Obstet Gynecol       Date:  2014-12-11

3.  Complete androgen insensitivity syndrome with accelerated onset of puberty due to a Sertoli cell tumor.

Authors:  Masako Izawa; Eiji Hisamatsu; Kaoru Yoshino; Makiko Yoshida; Takeshi Sato; Satoshi Narumi; Tomonobu Hasegawa; Takashi Hamajima
Journal:  Clin Pediatr Endocrinol       Date:  2021-04-03

4.  Malignant Leydig Cell Tumor in Elderly Complete Androgen Insensitivity Patient: A Case Report.

Authors:  Sundar Shrestha; Niroj Banepali; Rakesh Sthapit; Dipesh Agrawal
Journal:  JNMA J Nepal Med Assoc       Date:  2019 Mar-Apr       Impact factor: 0.406

  4 in total

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