An Adolescent Girl with Coexisting Ovarian Mature Cystic Teratoma and HAIR-AN Syndrome, an Extreme Subtype of Polycystic Ovarian Syndrome
February 2021
in “
Endocrinology, diabetes & metabolism case reports
”
HAIR-AN syndrome polycystic ovarian syndrome secondary amenorrhea virilizing signs frontal baldness clitoromegaly insulin resistance acanthosis nigricans testosterone mature cystic teratoma polycystic ovary laparoscopic cystectomy HOMA-IR hyperandrogenic symptoms androgen-secreting tumor PCOS baldness cystectomy androgen tumor
TLDR A teenage girl had both a rare ovarian tumor and a severe form of polycystic ovarian syndrome.
The document reported the case of a 15-year-old obese girl with secondary amenorrhea, virilizing signs including frontal baldness and clitoromegaly, and signs of insulin resistance such as marked acanthosis nigricans. Her total testosterone level was significantly elevated at 9.4 nmol/L, and an MRI revealed a right ovarian mass diagnosed as a mature cystic teratoma, as well as a left polycystic ovary. Following laparoscopic cystectomy, her testosterone levels decreased but remained high, and her menstrual cycle resumed but was irregular. Her fasting insulin level was also elevated at 85.2 mIU/L, and she had a high HOMA-IR of 13.1, indicating persistent insulin resistance. These findings suggested the coexistence of HAIR-AN syndrome, an extreme phenotype of PCOS, with an ovarian androgen-secreting tumor. The case emphasized that rapid onset of hyperandrogenic symptoms, particularly with virilization, should prompt consideration of an androgen-secreting tumor, and that significantly elevated testosterone levels may indicate a condition other than PCOS.
