Dysgerminoma in three patients with Swyer syndrome
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Dysgerminoma in three patients with Swyer syndrome Nadereh Behtash* and Mojgan Karimi Zarchi Address: Gynecology Oncology Department, Vali-Asr Hospital, Keshavarz Blvd., Tehran 14194, Iran Email: Nadereh Behtash* - [email protected]; Mojgan Karimi Zarchi - [email protected] * Corresponding author
Published: 23 June 2007 World Journal of Surgical Oncology 2007, 5:71
doi:10.1186/1477-7819-5-71
Received: 2 January 2007 Accepted: 23 June 2007
This article is available from: http://www.wjso.com/content/5/1/71 © 2007 Behtash and Karimi Zarchi; licensee BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
Abstract Background: Dysgerminoma is the most common malignant germ cell tumor of the ovary. This malignancy can be associated with pure gonadal dysgenesis or Swyer syndrome, mixed gonadal dysgenesis and partial gonadal dysgenesis. Case presentation: Dysgerminoma developed in 3 phenotypic female patients with 46 XY pure gonadal dysgenesis. All patients presented first with abdominopelvic mass. Laparatomy was done. 46 XY karyotype was made by lymphocyte culture. Then these patients underwent gonadectomy that histopathology results were streak ovaries without evidence for malignancy. Two patients received postoperative adjuvant therapy. Conclusion: In Patients with Swyer syndrome the risk of dysgerminoma is high and gonadectomy is recommended. Also 5% of dysgerminomas are discovered in phenotypic female and 46 XY karyotype, thus in adolescent with dysgerminoimas and amenorrhea, karyotype should be done.
Background Since 1955, when Swyer [1,2] first described two phenotypic women with gonadal dysgenesis without the stigma of Turner syndrome (46, XY pure gonadal dysgenesis of Swyer syndrome), several authors have reported over 74 tumoral growths of their dysgenetic gonads [3-19]. The propensity of tumor development in Swyer syndrome is significant, a incidence of 20–30% is reported. The most common tumor is the often-bilateral gonadoblastoma, but dysgerminoma and even embryonal carcinoma also seen [2]. Approximately 5% of dysgerminomas are discovered in phenotypic females with abnormal gonads and 46 XY karyotype [1]. We present three patients with pelvic mass, of which two patients, in spite of primary amenorrhea had nearly complete secondary sex characteristic. Another patient had
secondary amenorrhea. All the patients had dysgerminoma, that underwent unilateral salpingoopherectomy and two of these, received adjuvant therapy. Gonadectomy after diagnosis of XY karyotype was done.
Case presentation Case 1 A 20 years old girl (160 cm height and 57 kg weight) presented in September 1999 with 6 months history of lower abdominal pain and gradual distention of abdomen. Clinically, she was noted to have an abdominal mass extending 4 cm above umbilicus. She had
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