Case Report

Archives of Gynecology and Obstetrics

, Volume 263, Issue 3, pp 134-136

First online:

Virilizing adrenal adenoma and primary amenorrhea in a girl with adrenal hyperplasia

  • G. ForsbachAffiliated withDepartments of Endocrinology and Gynecology, Hospital de Gineco-Obstetricia, Dr. I. Morones, IMSS, Apartado Postal 71. CP 64000. Monterrey, NL, México e-mail: gefor@sysop.com.mx Tel.: +52-8-348-7409, Fax: +52-8-347-2021
  • , A. Güitrón-CantúAffiliated withDepartments of Endocrinology and Gynecology, Hospital de Gineco-Obstetricia, Dr. I. Morones, IMSS, Apartado Postal 71. CP 64000. Monterrey, NL, México e-mail: gefor@sysop.com.mx Tel.: +52-8-348-7409, Fax: +52-8-347-2021
  • , J. Vázquez-LaraAffiliated withDepartments of Endocrinology and Gynecology, Hospital de Gineco-Obstetricia, Dr. I. Morones, IMSS, Apartado Postal 71. CP 64000. Monterrey, NL, México e-mail: gefor@sysop.com.mx Tel.: +52-8-348-7409, Fax: +52-8-347-2021
  • , M. Mota-MoralesAffiliated withDepartments of Endocrinology and Gynecology, Hospital de Gineco-Obstetricia, Dr. I. Morones, IMSS, Apartado Postal 71. CP 64000. Monterrey, NL, México e-mail: gefor@sysop.com.mx Tel.: +52-8-348-7409, Fax: +52-8-347-2021
  • , M. L. Díaz-MendozaAffiliated withDepartments of Endocrinology and Gynecology, Hospital de Gineco-Obstetricia, Dr. I. Morones, IMSS, Apartado Postal 71. CP 64000. Monterrey, NL, México e-mail: gefor@sysop.com.mx Tel.: +52-8-348-7409, Fax: +52-8-347-2021

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Abstract 

We report a 14-year-old girl with primary amenorrhea and virilization. The chromosomal analysis showed a normal 46,XX female karyotype and the hormonal assays disclosed high serum levels of testosterone (T): 450 ng/dL (normal 5–90), dehidroepiandrosterone.sulfate (DHEA-S): 690 µg/dL (normal 30–450) and 17-hydroxiprogesterone (17-OHP) >20 ng/mL (normal <2). A pelvic ultrasound disclosed a small uterus and bilateral enlargement of the ovaries, a computed axial tomographic scan of the abdomen identified a large right mass in the adrenal gland and a norcholesterol-I 131 adrenal gammagraphy revealed a functional adrenal tumor. The histological analysis of the surgical removed tumor revealed and adrenal adenoma. After surgery, a steep decline to normal serum levels of T and DHEA-S was observed, remaining an elevated level of 17-OHP: 5.4 ng/mL. During the first three months of follow up, the hirsutism declined sharply and spontaneous mammary development occurred, remaining elevated the 17-OHP serum level: 4.8 ng/mL. Prednisone 5 mg/day, was initiated decreasing the 17-OHP to normal level: 1.4 ng/mL, appearing the menarche followed by cyclical menses. One year after surgery, prednisone was withdrawn during one week, and an ACTH test and HLA typing were done, disclosing a 17-OHP response of an heterozygote for adrenal hyperplasia, and identifying B65 a subtype of B14, and DR1, that are frequently associated to adrenal hyperplasia. Previous reports have informed silent adrenal tumors associated to adrenal hyperplasia, but this is the first report of a functional adrenal tumor associated to adrenal hyperplasia.

Key words Virilization Primary amenorrhea Functional adrenal adenoma Puberty Adrenal hyperplasia