Gonadal dysgenesis and gynecologic cancer.
BACKGROUND
Gonadal dysgenesis encompasses a variety of sexual differentiation disorders. Within this population of patients, there is an increased risk of gonadal tumor formation.


METHODS
In this case series of three patients, two with Swyer's syndrome (complete gonadal dysgenesis) and one with mosaic Turner's syndrome, three separate histologic subtypes of tumors were identified: dysgerminoma, seminoma, and gonadoblastoma. The patients with dysgerminoma and seminoma had regular menses and were without recurrent disease. We recommend that the patient with gonadoblastoma start on hormone therapy.


CONCLUSIONS
Once the diagnosis of gonadal dysgenesis is made, prophylactic gonadectomy should be performed owing to the probability of malignant transformation. These patients illustrate the potential different presentations with gonadal dysgenesis and the importance of complete evaluation of patients with primary amenorrhea.
Jonson
Amy L
AL
From the Division of Gynecologic Oncology, University of Minnesota Department of Obstetrics, Gynecology and Women's Health, Minneapolis, Minnesota.
Geller
Melissa A
MA
Dickson
Elizabeth L
EL
D000008:Abdominal Neoplasms / Q000209:etiology; D000293:Adolescent; D000568:Amenorrhea / Q000209:etiology; D004407:Dysgerminoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D005260:Female; D006059:Gonadal Dysgenesis / Q000150:complications*; D006061:Gonadal Dysgenesis, 46,XY / Q000150:complications; D018238:Gonadoblastoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D006801:Humans; D010051:Ovarian Neoplasms / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D018239:Seminoma / Q000209:etiology / Q000473:pathology* / Q000601:surgery; D014424:Turner Syndrome / Q000150:complications; D055815:Young Adult