Dysgerminoma in three patients with Swyer syndrome

نویسندگان

  • Nadereh Behtash
  • Mojgan Karimi Zarchi
چکیده

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.

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منابع مشابه

Incidental gonadal tumors at the time of gonadectomy in women with Swyer syndrome: a case series.

BACKGROUND Swyer syndrome (46XY complete gonadal dysgenesis) is an uncommonly encountered condition in our population. Gonadectomy is recommended upon diagnosis due to a significant risk of malignant transformation of the dysgenetic gonads, typically to dysgerminoma. CASES We present 3 cases of women who underwent gonadectomy following a diagnosis of Swyer syndrome. Two of these patients had ...

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A Case of Swyer Syndrome Associated with Advanced Gonadal Dysgerminoma Involving Long Survival

Swyer syndrome is caused by abnormal sex differentiation during the embryonic period, resulting in incomplete intrauterine masculinization and undifferentiated gonads. The current case report describes a patient with Swyer syndrome associated with stage 3 gonadal dysgerminoma who has survived for 23 years. At age 18, this patient sought assistance for primary amenorrhea from the Gynecological S...

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Dysgerminoma in a case of 46, XY pure gonadal dysgenesis (swyer syndrome): a case report

Simple 46, XY gonadal dysgenesis syndrome, also called Swyer syndrome, is known as pure gonadal dysgenesis. Individuals with the syndrome are characterized by 46, XY karyotype and phenotypically female with female genital appearance, normal Müllerian structures and absent testicular tissue. The condition usually first becomes apparent in adolescence with delayed puberty and primary amenorrhea d...

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Swyer syndrome: presentation and outcomes.

OBJECTIVE To establish the spectrum of presentation, natural history and gynaecological outcomes in women with Swyer syndrome. DESIGN Retrospective notes review. SETTING Tertiary referral centre for disorders of sex development. POPULATION A total of 29 adult women with Swyer syndrome. METHODS Information was collected on age at diagnosis, biometric characteristics, timing of gonadectom...

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عنوان ژورنال:
  • World Journal of Surgical Oncology

دوره 5  شماره 

صفحات  -

تاریخ انتشار 2007