Primary amenorrhea revealing an occult progesterone-secreting ovarian tumor.
OBJECTIVE
To describe a patient with primary amenorrhea revealing an occult progesterone-secreting ovarian tumor.


METHODS
Case report.


METHODS
University medical center.


METHODS
A 20-year-old woman with primary amenorrhea.


METHODS
Investigations to identify the source of progesterone secretion.


METHODS
Discovery of an occult progesterone-secreting ovarian tumor.


RESULTS
Initial ovarian ultrasonography did not show any abnormal mass. Catheterization of ovarian veins suggested a right ovarian source of progesterone. After long-term follow-up, a right ovarian tumor became apparent and was surgically removed. After surgery, progesterone levels decreased and normal ovulatory cycles resumed. Pathologic and immunohistochemical analysis showed a Leydig cell tumor expressing cytochrome P450 side-chain cleavage and 3ss-hydroxysteroïd dehydrogenase enzymes, which are involved in progesterone biosynthesis, whereas P45017alpha-hydroxylase was not expressed, explaining the absence of hyperandrogenemia. Before surgery, two LH pulses were detected during a 6-hour study period and a lack of ovarian response to pulsatile GnRH administration.


CONCLUSIONS
This is the first case of isolated progesterone secretion by an occult ovarian Leydig cell tumor and a novel etiology of primary amenorrhea. The results also suggest that sustained progesterone can exert an inhibitory effect on gonadotropin secretion at both hypothalamic and pituitary levels.
Bry-Gauillard
Helene
H
Service d'Endocrinologie et des Maladies de la Reproduction, Universite Paris-Sud 11, Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Le Kremlin Bicêtre Le Kremlin Bicêtre, France.
Meduri
Geri
G
Abirached
Fadi
F
Constancis
Elisabeth
E
Brailly
Sylvie
S
Chanson
Philippe
P
Young
Jacques
J
D000328:Adult; D000568:Amenorrhea / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D033162:Incidental Findings; D009382:Neoplasms, Unknown Primary / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism* / Q000628:therapy; D010051:Ovarian Neoplasms / Q000150:complications / Q000175:diagnosis* / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D011374:Progesterone / Q000378:metabolism*
D011374:Progesterone