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Updated: Jul 7, 2026
A Coregistered Ultrasound and Photoacoustic Imaging Protocol for the Transvaginal Imaging of Ovarian Lesions
Published on: March 3, 2023
Primary amenorrhea revealing an occult progesterone-secreting ovarian tumor
Helene Bry-Gauillard1, Geri Meduri, Fadi Abirached
1Service 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.
Objective:
To describe a patient with primary amenorrhea revealing an occult progesterone-secreting ovarian tumor.
Design:
Case report.
Setting:
University medical center.
Patient(S):
A 20-year-old woman with primary amenorrhea.
Intervention(S):
Investigations to identify the source of progesterone secretion.
Main Outcome Measure(S):
Discovery of an occult progesterone-secreting ovarian tumor.
Result(S):
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.
Conclusion:
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.
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