1527 GONADAL TUMOR DETECTION AND TREATMENT IN PATIENTS WITH DISORDER OF SEX DEVELOPMENT (DSD) LONG-TERM ONCOLOGICAL OUTCOMES

Abstract

You have accessJournal of UrologyPediatrics: Congenital Anomalies - Lower Urinary Tract & Genitalia I1 Apr 20121527 GONADAL TUMOR DETECTION AND TREATMENT IN PATIENTS WITH DISORDER OF SEX DEVELOPMENT (DSD) LONG-TERM ONCOLOGICAL OUTCOMES Lorena Oliveira, Marcos Machado, Giovanni Marchini, Alessandro Tavares, Maria Helena Sircili, Sorahia Domenice, Elaine Costa, Francisco Denes, Berenice Mendonça, and Miguel Srougi Lorena OliveiraLorena Oliveira Sao Paulo, Brazil More articles by this author , Marcos MachadoMarcos Machado Sao Paulo, Brazil More articles by this author , Giovanni MarchiniGiovanni Marchini Sao Paulo, Brazil More articles by this author , Alessandro TavaresAlessandro Tavares Sao Paulo, Brazil More articles by this author , Maria Helena SirciliMaria Helena Sircili Sao Paulo, Brazil More articles by this author , Sorahia DomeniceSorahia Domenice Sao Paulo, Brazil More articles by this author , Elaine CostaElaine Costa Sao Paulo, Brazil More articles by this author , Francisco DenesFrancisco Denes Sao Paulo, Brazil More articles by this author , Berenice MendonçaBerenice Mendonça Sao Paulo, Brazil More articles by this author , and Miguel SrougiMiguel Srougi Sao Paulo, Brazil More articles by this author View All Author Informationhttps://doi.org/10.1016/j.juro.2012.02.1295AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookTwitterLinked InEmail INTRODUCTION AND OBJECTIVES To expose a 18-year experience in the management of DSD patients with gonadal tumors (GT). METHODS From 1993 to 2011, 14 DSD patients were found to have GT. Mean age at diagnosis was 25.4 ± 10.8 (6.8-43.7) years and social sex was female in 9. Clinical diagnosis was Turner's syndrome in 2, gonadal dysgenesis in 7 and Frasier syndrome, complete and partial androgen insensivity syndrome, 17-beta-hydroxysteroid dehydrogenase deficiency and Mullerian inhibiting factor deficiency in 1 each. Karyotype was 46XY in 11, 45X/46XY in 2 and 46XY/47XYY in 1. Mean follow-up time was 96.2 ± 56.7 (21.1–186.9) months. RESULTS Diagnosis was made by preoperative evaluation or as surgical finding. Laparoscopic gonadectomy was performed in 8 patients. Open gonadal/ductal structures resection was performed by inguinotomy in 2 and through an abdominal incision in 4 patients. Of these 6 patients, 2 underwent laparoscopic excision of remanescent intra-abdominal structures. Pathologic examination revealed gonadoblastoma associated with intratubular neoplasia in 6 patients (3 bilateral), 2 dysgerminomas, 2 seminomas, 2 non-seminomas, 1 Leydig and 1 Sertoli cel tumor. One patient with dysgerminoma and one with non-seminoma underwent adjuvant chemotherapy. No patient had disease recurrence and overall survival was 100%. CONCLUSIONS GT in patients with DSD are clinically unapparent and demand accurate investigation and precise diagnosis. Laparoscopic evaluation of intra-abdominal structures allows therapeutic or prophylactic resection whenever advised. Gonadectomy offers exceptional oncological outcomes in this population. © 2012 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 187Issue 4SApril 2012Page: e618 Peer Review Report Advertisement Copyright & Permissions© 2012 by American Urological Association Education and Research, Inc.MetricsAuthor Information Lorena Oliveira Sao Paulo, Brazil More articles by this author Marcos Machado Sao Paulo, Brazil More articles by this author Giovanni Marchini Sao Paulo, Brazil More articles by this author Alessandro Tavares Sao Paulo, Brazil More articles by this author Maria Helena Sircili Sao Paulo, Brazil More articles by this author Sorahia Domenice Sao Paulo, Brazil More articles by this author Elaine Costa Sao Paulo, Brazil More articles by this author Francisco Denes Sao Paulo, Brazil More articles by this author Berenice Mendonça Sao Paulo, Brazil More articles by this author Miguel Srougi Sao Paulo, Brazil More articles by this author Expand All Advertisement Advertisement PDF downloadLoading ...