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Question 30#

A 32-year-old woman has a 3-year history of oligomenorrhea that has progressed to amenorrhea during the past year. She has observed loss of breast fullness, reduced hip measurements, acne, increased body hair, and deepening of her voice. Physical examination reveals frontal balding, clitoral hypertrophy, and a male escutcheon. Urinary free cortisol and dehydroepiandrosterone sulfate (DHEAS) are normal. Her plasma testosterone level is 6 ng/mL (normal is 0.2-0.8). Which of the following is the most likely diagnosis?

a. Cushing syndrome
b. Arrhenoblastoma
c. Polycystic ovary syndrome
d. Granulosa-theca cell tumor
e. Ovarian teratoma

Correct Answer is B

Comment:

The symptoms of masculinization (eg, alopecia, deepening of voice, clitoral hypertrophy) in this patient are characteristic of an active androgen-producing tumor. Such extreme virilization is very rarely observed in polycystic ovary syndrome or in Cushing syndrome; moreover, the presence of normal cortisol and adrenal androgens (DHEA-S) plus markedly elevated plasma testosterone levels indicates an ovarian rather than adrenal cause of the findings. Arrhenoblastomas are the most common androgen-producing ovarian tumors. Their incidence is highest during the reproductive years. Composed of varying proportions of Leydig and Sertoli cells, they are generally benign. In contrast to arrhenoblastomas, granulosa-theca cell tumors produce feminization, not virilization. Dermoid cysts (benign teratomas) do not produce gonadotropins but cause symptoms by enlargement, ovarian torsion (pain) or rupture with contents spilling into the peritoneal cavity.