Gonadal malignancy in 202 female patients with disorders of sex development containing Y-chromosome material
- 16 November 2015
- journal article
- research article
- Published by Informa UK Limited in Gynecological Endocrinology
- Vol. 32 (4), 338-341
- https://doi.org/10.3109/09513590.2015.1116509
Abstract
The objective of this study was to examine risks for gonadal malignancy in a large sample of adult female patients with disorders of sex development (DSD). A retrospective-observational study was conducted from July 1992 to March 2015 and 202 women with DSD were enrolled. Tumor risks for different types of DSD were measured. We found that the patients’ total gonadal-malignancy risk was 18.3% (37/202). Tumors included gonadoblastoma (n = 11), seminoma (n = 8), dysgerminoma (n = 5), choriocarcinoma (n = 1), sertoli cell tumors (n = 11), and leydig cell tumors (n = 1). The incidence of gonadal malignancy in patients with complete androgen insensitivity syndrome (CAIS), pure 46, XY gonadal dysgenesis, 45 X/46 XY mixed gonadal dysgenesis, 17α-hydroxylase/17, 20-lyase deficiency and partial androgen insensitivity syndrome (PAIS) were 27.1% (13/48), 22.4% (15/67), 10.9% (5/46), 10% (2/20) and 9.5% (2/21), respectively. Our results suggest that the incidence of gonadal malignancy increases with age for female patients with Y-chromosome material. Upon diagnoses, immediate, prophylactic gonadectomies should be considered for adult female patients with DSD containing Y chromosome material if they cannot receive regular follow-ups.Keywords
This publication has 25 references indexed in Scilit:
- Gonadal malignancy risk and prophylactic gonadectomy in disorders of sexual developmentJournal of Pediatric Endocrinology and Metabolism, 2015
- Gonadal malignancy in 13 consecutive collected patients with disorders of sex development (DSD) from Semarang (Indonesia)Journal of Clinical Pathology, 2012
- Patients with disorders of sex development (DSD) at risk of gonadal tumour development: management based on laparoscopic biopsy and molecular diagnosisBJU International, 2012
- Delayed Recognition of Disorders of Sex Development (DSD): A Missed Opportunity for Early Diagnosis of Malignant Germ Cell TumorsInternational Journal of Endocrinology, 2012
- Tumor Risk in Disorders of Sex DevelopmentSexual Development, 2010
- Gonadal tumours and DSDBest Practice & Research Clinical Endocrinology & Metabolism, 2010
- Tumor risk in disorders of sex development (DSD)Best Practice & Research Clinical Endocrinology & Metabolism, 2007
- Germ Cell Tumors in the Intersex Gonad: Old Paths, New Directions, Moving FrontiersEndocrine Reviews, 2006
- Consensus statement on management of intersex disordersArchives of Disease in Childhood, 2005
- Abnormal sexual differentiation and neoplasiaCancer Genetics and Cytogenetics, 1987