[Bilateral Leydig cell tumor of the ovary in a woman with congenital adrenal hyperplasia. The first reported case].
A 26-year-old woman with congenital adrenal hyperplasia (CAH) due to 11 hydroxylase deficiency complained of infertility. Clinical examination disclosed no evolutive virilizing features. Basal serum levels of delta 4 androstenedione and testosterone, under hydrocortisone suppressive therapy, where elevated at 4.72 and 2.84 ng/ml respectively (N: 1.2 to 2.2 and 0.2 to 0.6) leading to the discovery of a right ovarian tumor. Post-operative evaluation (8 months later) revealed dexamethasone-sensitive hyperandrogenism and a coincidental large left ovarian tumor. Three months after the second surgical procedure, the patient became pregnant and gave birth to a non-virilized girl. At microscopic examination both tumors were typical Leydig cell tumors of the ovary containing crystals of Reinke. This is the first case of bilateral and macroscopic Leydig cell tumor ot the ovary in a woman with congenital adrenal hyperplasia. Relationships between hilus cell tumors and steroid cell tumors of the adrenocortical type are discussed.
Luton
J P
JP
Clinique des Maladies Endocriniennes et Métaboliques, Hôpital Cochin, Paris.
Clerc
J
J
Paoli
V
V
Bonnin
A
A
Dumez
Y
Y
Vacher-Lavenu
M C
MC
D000312:Adrenal Hyperplasia, Congenital / Q000150:complications*; D000328:Adult; D005260:Female; D006801:Humans; D007984:Leydig Cell Tumor / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D010051:Ovarian Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography
Tumeur ovarienne bilatérale à cellules de Leydig-Berger chez une femme ayant une hyperplasie congénitale des surrénales. Le premier cas rapporté.