Journal of Andrology
HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH
Published-Ahead-of-Print August 15, 2007, DOI:10.2164/jandrol.107.003053

This Article
Right arrow Author Manuscript (PDF)
Right arrow All Versions of this Article:
29/1/15    most recent
Author Manuscript (PDF)
Right arrow Alert me when this article is cited
Right arrow Alert me if a correction is posted
Services
Right arrow Similar articles in this journal
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Download to citation manager
Citing Articles
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by Goglia, U.
Right arrow Articles by Ferone, D.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by Goglia, U.
Right arrow Articles by Ferone, D.

A unique association of clinical "Persistent Mullerian Duct Syndrome" and syringoid carcinoma of the perineal-scrotal skin: a consequence of urological surgery?

Umberto Goglia , Carlo Toncini , Massimo Giusti , Christian Gastaldi , Carlo Ambruosi , Simona Sola , Jean-Luis Ravetti , Giorgio Carmignani , Francesco Minuto , and Diego Ferone *

* To whom correspondence should be addressed. E-mail: ferone{at}unige.it.

Persistent Mullerian Duct Syndrome (PMDS) is a rare form of male pseudohermaphroditism occurring in 46 XY males, and is caused by a defect in either the gene for the Mullerian inhibiting substance (MIS) or its type 2 receptor. Patients with this syndrome present retained Mullerian ducts and unilateral or bilateral undescended testes, and may also display crossed testicular ectopia caused by herniated uterine structures (MacLaughlin et al, 2004; Belville et al, 2004). Sexual ambiguity, PMDS particularly, is also present in other species, such as mixbred dogs and goats (Josso et al, 2005; Kuiper et al, 2004; Meyers-Wallen et al,1993). In this paper we report the history of a 46-years-old man with a scrotal mass, diagnosed as syringoid eccrine carcinoma, presenting a PMDS, incidentally showed by MRI performed for pre-surgery stadiation of the scrotal tumor. To our knowledge this is the first case of an exceptional association between an unusual form of male pseudohermaphroditism and a rare cutaneous tumor in an unusual site, probably due to a previous reconstructive surgery for hypospadia.


Key words: Intersex • Surgery • Anti-Mullerian Hormone • Persistent Müllerian Duct Syndrome • eccrine carcinoma • male pseudohermaphroditism






HOME HELP FEEDBACK SUBSCRIPTIONS ARCHIVE SEARCH
Copyright © 2007 by The American Society of Andrology.