A 33-year-old woman comes to the office due to new-onset pain with intercourse. The patient has been in a relationship with a male partner for the past 6 months and is using condoms for contraception. For the past 3 months, she has had worsening dyspareunia and vaginal dryness, which has made her limit sexual activity despite no changes in libido. She has had 6 lifetime partners and has not had this problem before. The patient has had no postcoital spotting or abnormal vaginal discharge. Family history is significant for a sister with infertility due to 45,X mosaicism. Vital signs are normal. Bilateral breasts are atrophic. On pelvic examination, the vulvae are pale and thin, and the clitoris protrudes from the clitoral hood. There is no tenderness to palpation over the perineum. The vagina has minimal rugation, and a large, mildly tender adnexal mass is palpated. Pregnancy test is negative. Pelvic ultrasound reveals a 9-cm, solid adnexal mass. Which of the following is the most likely etiology of this patient's presentation?

Question

A 33-year-old woman comes to the office due to new-onset pain with intercourse.  The patient has been in a relationship with a male partner for the past 6 months and is using condoms for contraception.  For the past 3 months, she has had worsening dyspareunia and vaginal dryness, which has made her limit sexual activity despite no changes in libido.  She has had 6 lifetime partners and has not had this problem before.  The patient has had no postcoital spotting or abnormal vaginal discharge.  Family history is significant for a sister with infertility due to 45,X mosaicism.  Vital signs are normal.  Bilateral breasts are atrophic.  On pelvic examination, the vulvae are pale and thin, and the clitoris protrudes from the clitoral hood.  There is no tenderness to palpation over the perineum.  The vagina has minimal rugation, and a large, mildly tender adnexal mass is palpated.  Pregnancy test is negative.  Pelvic ultrasound reveals a 9-cm, solid adnexal mass.  Which of the following is the most likely etiology of this patient's presentation?

Quizplus · Accepted Answer

Explanation: 11ec25c8_d9d4_039a_bc81_afe5e03c75ef_MD0003_00 This patient's clitoromegaly (eg, clitoris protruding from the clitoral hood) and large adnexal mass are most likely due to a Sertoli-Leydig cell tumor, a testosterone-secreting sex cord-stromal tumor. Sertoli and Leydig cells are normally found in the testes but can develop in the ovaries and produce testosterone, particularly after malignant transformation and cell proliferation. The testosterone excess results in the clinical features often associated with this tumor, including: Rapid-onset virilization: Testosterone and dihydrotestosterone affect peripheral tissues, resulting in clitoromegaly, as seen in this patient. Additional features may include male-pattern (bitemporal) balding, voice deepening, and increased muscle mass. Signs of estrogen deficiency: Testosterone inhibits hypothalamic GnRH and pituitary FSH/LH release, resulting in low estrogen. Therefore, patients may develop breast atrophy, vulvovaginal atrophy, dyspareunia, and oligomenorrhea. Sertoli-Leydig cells are typically diagnosed at an early cancer stage, and management includes surgical removal. Those with metastatic disease may require additional chemotherapy. (Choice A) Turner syndrome (45,X) causes chromosome-mediated ovarian follicle depletion, resulting in primary ovarian insufficiency. These patients have signs of estrogen deficiency (eg, dyspareunia, vaginal atrophy); however, they have no associated virilization (eg, clitoromegaly) or adnexal mass, making this diagnosis unlikely. (Choice B) A tubo-ovarian abscess is a complex collection of purulent, polymicrobial fluid that can present as a complex adnexal mass. The abscess typically manifests acutely with fever, and patients have no associated virilization. (Choice C) Endometriomas (endometrial glands and stroma within the ovary) can present as complex adnexal masses. Patients with endometriosis may have dyspareunia but no associated virilization or estrogen deficiency. (Choice D) Granulosa cell tumors (estrogen-secreting sex cord-stromal tumors) present as large, complex ovarian masses. In contrast to this patient, those with a granulosa cell tumor have signs of elevated estrogen (eg, breast tenderness) and normal vaginal rugation. (Choice E) Lactate dehydrogenase-secreting germ cell tumors (eg, dysgerminomas) cause solid adnexal masses. Dysgerminomas may secrete hCG and estrogen but not testosterone, making this diagnosis unlikely. (Choice F) Serous epithelial ovarian tumors are a common type of ovarian cancer; these patients have no associated virilization. Educational objective: A Sertoli-Leydig cell tumor, a sex cord-stromal tumor, is a testosterone-secreting ovarian tumor. Patients can have virilization (eg, clitoromegaly) and signs of estrogen deficiency (eg, breast atrophy, vulvovaginal atrophy). References: Fertility-sparing management and obstetric outcomes in a 20-year-old patient with a Sertoli-Leydig cell tumor of the ovary: a case report and review of the literature. (http://www.ncbi.nlm.nih.gov/pubmed/27446397) Clinicopathologic features of ovarian Sertoli-Leydig cell tumors. (http://www.ncbi.nlm.nih.gov/pubmed/25400781) Ovarian Sertoli-Leydig cell tumours: how typical is their typical presentation? (http://www.ncbi.nlm.nih.gov/pubmed/28485183)

A 33-Year-Old Woman Comes to the Office Due to New-Onset