Document Detail

Complete androgen insensitivity syndrome associated with bilateral sertoli cell adenomas and paratesticular leiomyomas: Case report and review of the literature.
MedLine Citation:
PMID:  22819761     Owner:  NLM     Status:  Publisher    
BACKGROUND: Complete androgen insensitivity syndrome (CAIS) is a rare and usually unexpected cause of primary amenorrhoea that results from receptor resistance to androgens, producing a female phenotype in genetically male patients. CASE: A 16-year-old girl was diagnosed with CAIS after investigations for primary amenorrhoea. Her left inguinal gonad and the right intra-abdominal gonad were resected and histopathology revealed the presence of dysgenetic multinodular testes with absence of germ cells, significant hyperplasia of Sertoli cells (Sertoli cell adenoma) and presence of paratesticular leiomyomas. CONCLUSION: Although the risk of gonadal tumour development is considered to be low, a variety of tumours have been described in association with CAIS, but this is the first report of development of bilateral paratesticular leiomyomas developing concurrently with Sertoli cell adenomas.
S Siminas; G Kokai; S E Kenny
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-7-20
Journal Detail:
Title:  Journal of pediatric urology     Volume:  -     ISSN:  1873-4898     ISO Abbreviation:  -     Publication Date:  2012 Jul 
Date Detail:
Created Date:  2012-7-23     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101233150     Medline TA:  J Pediatr Urol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
Alder Hey Children's NHS Foundation Trust, Department of Paediatric Surgery and Urology, UK.
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