Clinical features and hormonal characteristics in a case of ovarian arrhenoblastoma.
We report a case of a 34-year-old woman affected with ovarian arrhenoblastoma characterized by very high testosterone (T) levels (34.0-60.0 ng/ml; n.v.0.2-0.9) and suppressed gonadotropin levels. The physical examination revealed: severe hirsutism, acne, amenorrhea and other virilization signs. Basal hormonal evaluation also showed a markedly elevated 17-hydroxyprogesterone (17-OHP) and a mild delta 4 Androstenedione (A) and dehydroepiandrosterone sulfate (DHEAs) increase. ACTH test induced only slight changes in androgen secretion. By contrast, dexamethasone test greatly decreased A and DHEAs whereas T levels were only partially suppressed. Moreover, hCG test was clearly stimulatory for T and A. Suppressed gonadotropin levels did not respond to LHRH stimulation. The removal of the neoplasia was followed by normalization of T levels and increase of serum gonadotropins with subsequent restoration of a normal responsiveness to LHRH and resumption of an ovulatory menstrual cycle. This observation suggests that the high T levels played a primary role in the pathogenesis of the gonadotropin suppression and anovulation. Recovery of acne was complete whereas hirsutism score was reduced but still elevated after one year. This may be due to postoperative A and DHEAs levels slightly above the normal range, indicating the presence of adrenal hyperandrogenism.
Tita
P
P
Cattedra e Divisione di Endocrinologia, Università di Catania, Ospedale Garibaldi, Catania, Italy.
Spina
A
A
Briguglia
G
G
Magro
A
A
Gallo
D
D
Finocchiaro
C
C
Padova
G
G
Pezzino
V
V
D019326:17-alpha-Hydroxyprogesterone / Q000097:blood; D000328:Adult; D000735:Androstenedione / Q000097:blood; D003687:Dehydroepiandrosterone / Q000097:blood; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D006065:Gonadotropins, Pituitary / Q000097:blood*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D007986:Luteinizing Hormone / Q000097:blood; D010051:Ovarian Neoplasms / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy; D018310:Sertoli-Leydig Cell Tumor / Q000503:physiopathology* / Q000601:surgery / Q000628:therapy; D013256:Steroids / Q000097:blood*; D013739:Testosterone / Q000097:blood
D006065:Gonadotropins, Pituitary; D013256:Steroids; D013739:Testosterone; D000735:Androstenedione; D003687:Dehydroepiandrosterone; D019326:17-alpha-Hydroxyprogesterone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone; D006854:Hydrocortisone