TY - JOUR
T1 - Gonadal dysgenesis and gynecologic cancer
AU - Jonson, Amy L.
AU - Geller, Melissa A.
AU - Dickson, Elizabeth L.
PY - 2010/8
Y1 - 2010/8
N2 - Background: Gonadal dysgenesis encompasses a variety of sexual differentiation disorders. Within this population of patients, there is an increased risk of gonadal tumor formation. Cases: 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. Conclusion: 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.
AB - Background: Gonadal dysgenesis encompasses a variety of sexual differentiation disorders. Within this population of patients, there is an increased risk of gonadal tumor formation. Cases: 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. Conclusion: 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.
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U2 - 10.1097/AOG.0b013e3181e4bfe9
DO - 10.1097/AOG.0b013e3181e4bfe9
M3 - Article
C2 - 20664451
AN - SCOPUS:77955155871
SN - 0029-7844
VL - 116
SP - 550
EP - 552
JO - Obstetrics and gynecology
JF - Obstetrics and gynecology
IS - 2 PART 2
ER -