J Korean Med Sci.  1993 Oct;8(5):380-384. 10.3346/jkms.1993.8.5.380.

Gonadoblastoma and dysgerminoma associated with 46,XY pure gonadal dysgenesis--a case report

Affiliations
  • 1Department of Obstetrics and Gynecology, Yonsei University College of Medicine, Seoul, Korea.

Abstract

Gonadoblastoma and dysgerminoma developed in a 24-year-old phenotypic female patient with 46,XY pure gonadal dysgenesis. This patient presented with primary amenorrhea. Clinical characteristics showed a typical stigmata of gonadal dysgenesis: primary amenorrhea, sexual infantilism, a small uterus and bilateral streak gonads. A 46,XY karyotype was made by lymphocyte culture. The patient was counseled to undergo a prophylactic bilateral gonadectomy, but she refused. Three years and three months after the initial diagnosis she felt a growing pelvic mass. Bilateral gonadectomy and total hysterectomy were performed. Histological examination revealed gonadoblastoma and dysgerminoma on both gonads. After surgery the patient received radiation therapy and also was started on hormone replacement therapy. Two years and two months after treatment by surgery the patient is well and free of recurrence.

Keyword

Gonadoblastoma; Dysgerminoma; 46; XY Pure Gonadal Dysgenesis; Gonadectomy

MeSH Terms

Adult
Dysgerminoma/*etiology/pathology/therapy
Female
Gonadal Dysgenesis, 46,XY/*complications
Gonadoblastoma/*etiology/pathology/therapy
Humans
Ovarian Neoplasms/*etiology/pathology/therapy

Cited by  1 articles

A Case of 46,XY Pure Gonadal Dysgenesis with Loss of the Sex-Determining Region of Y Chromosome
Kui hyun Yoon, Young Jin Lee
Korean J Lab Med. 2008;28(2):151-154.    doi: 10.3343/kjlm.2008.28.2.151.

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