| 46,XY pure gonadal dysgenesis: Clinical presentations and management of the tumor risk. | |
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MedLine Citation:
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PMID: 20153258 Owner: NLM Status: In-Data-Review |
Abstract/OtherAbstract:
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PATIENTS: Eleven patients with 46,XY PGD were divided into two groups. Six symptomatic girls (group 1) were referred for amenorrhea (n = 3), gonadal tumor (n = 2) or campomelic dysplasia (n = 1). Five asymptomatic screened patients (group 2) were diagnosed as 46,XY PGD after familial investigation of the two probands with gonadal tumor. Bilateral gonadectomy was performed in all patients. RESULTS: In group 1, pathologic examination revealed an association of dysgerminoma with gonadoblastoma (n = 2), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). Prophylactic gonadectomy in asymptomatic patients (group 2) also showed asymptomatic dysgerminoma with gonadoblastoma (n = 1), bilateral gonadoblastoma (n = 2) and streak gonads (n = 2). CONCLUSIONS: A gonadal tumor arising in a girl with pubertal delay may be related to dysgenesis of the gonad. Primary amenorrhea or diagnosis of dysgerminoma should warrant karyotype, and familial study if 46,XY PGD is found. Considering the high incidence of gonadoblastoma and the early occurrence of dysgerminoma, early bilateral gonadectomy is recommended. |
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Authors:
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Carmen Capito; Marc-David Leclair; Alexis Arnaud; Albert David; Sabine Baron; Nadège Corradini; Yves Héloury |
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Publication Detail:
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Type: Journal Article Date: 2010-02-11 |
Journal Detail:
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Title: Journal of pediatric urology Volume: 7 ISSN: 1873-4898 ISO Abbreviation: J Pediatr Urol Publication Date: 2011 Feb |
Date Detail:
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Created Date: 2011-01-18 Completed Date: - Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 101233150 Medline TA: J Pediatr Urol Country: England |
Other Details:
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Languages: eng Pagination: 72-5 Citation Subset: IM |
Copyright Information:
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Copyright © 2010 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved. |
Affiliation:
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Department of pediatric surgery, Hopital Mère-Enfant, Nantes, France. |
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From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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