title: Primary amenorrhea revealing an occult progesterone-secreting ovarian tumor.
abstract: 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.
authors.affiliation: 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.
authors.forename: Helene
authors.initials: H
authors.lastname: Bry-Gauillard
authors.forename: Geri
authors.initials: G
authors.lastname: Meduri
authors.forename: Fadi
authors.initials: F
authors.lastname: Abirached
authors.forename: Elisabeth
authors.initials: E
authors.lastname: Constancis
authors.forename: Sylvie
authors.initials: S
authors.lastname: Brailly
authors.forename: Philippe
authors.initials: P
authors.lastname: Chanson
authors.forename: Jacques
authors.initials: J
authors.lastname: Young
mesh_terms: 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*
chemical_list: D011374:Progesterone