This patient with an ovarian mass, hirsutism (eg, coarse facial hair), and voice deepening likely has a Sertoli-Leydig cell tumor, an ovarian sex cord–stromal tumor.  Ovarian tumors can be associated with increased hormonal or enzymatic activity based on their cell composition.  Sertoli and Leydig cells are normally found in the male testes but can develop in the ovaries and produce excess testosterone.  With proliferation of these cells, male-range testosterone levels can occur and cause signs of hyperandrogenism.  Therefore, patients typically present with:

• Frank virilization:  Testosterone excess can cause hirsutism and clitoromegaly, as seen in this patient.  Additional features may include male pattern (bitemporal) baldness, voice deepening, and increased muscle mass.

• Anovulation and amenorrhea:  Elevated testosterone levels suppress hypothalamic GnRH and pituitary FSH/LH release.  Therefore, patients may develop signs of anovulation such as abnormal uterine bleeding or amenorrhea.

Diagnosis is supported by markedly elevated serum testosterone levels, but surgical removal is needed for definitive histologic diagnosis.

(Choices A and B)  Dysgerminomas and endodermal sinus (yolk sac) tumors are both malignant ovarian germ cell tumors with hormonal activity.  Dysgerminomas secrete β-hCG and lactate dehydrogenase; yolk sac tumors secrete alpha-fetoprotein.  Because they do not secrete testosterone, neither tumor type is associated with rapid-onset virilization.

(Choice C)  Granulosa cell tumors are sex cord–stromal tumors that secrete estrogen and inhibin.  Patients typically have signs of excessive estrogen (eg, breast tenderness, abnormal uterine bleeding due to endometrial hyperplasia) rather than signs of excess testosterone.

(Choice D)  Mature cystic teratomas (dermoid cysts) are the most common benign germ cell tumor and are typically composed of several differentiated tissues (eg, hair, sebaceous glands) arising from the ectoderm, mesoderm, and endoderm.  Most mature cystic teratomas do not produce hormones.  The exception is struma ovarii, a monodermal teratoma mostly composed of thyroid tissue that can secrete thyroid hormone and cause signs of hyperthyroidism (eg, tachycardia, weight loss).

(Choice E)  Serous cystadenocarcinomas are the most common epithelial ovarian cancer but typically have no hormonal or enzymatic activity.

Educational objective:
Sertoli-Leydig cell tumors of the ovary arise from the sex cord stroma and secrete testosterone.  Therefore, patients typically have signs and symptoms of hyperandrogenism, including rapid-onset virilization (eg, hirsutism, clitoromegaly, voice deepening) and amenorrhea.