A 38-year-old woman, gravida 3 para 3, comes to the office for evaluation of abnormal uterine bleeding. The patient had regular, monthly menses until 6 months ago, when she began to have intermenstrual spotting and has now been amenorrheic for 3 months. She has had increased abdominal bloating and cramping during the last few months but no hot flushes or weight changes. The patient has no chronic medical conditions and underwent a bilateral tubal ligation after her last delivery 2 years ago. She takes no daily medications and does not use tobacco, alcohol, or illicit drugs. Blood pressure is 124/70 mm Hg and pulse is 88/min. BMI is 29 kg/m². There is coarse hair on the upper lip and bitemporal hair thinning. Examination reveals a large pelvic mass extending throughout the left lower quadrant, but no tenderness or ascites. The cervix appears multiparous with no active bleeding. Urine pregnancy test is negative. Which of the following is the most likely diagnosis in this patient?

Question

Quizplus · Accepted Answer

11ec25c8_d9e1_712f_bc81_7905b713e696_MD0003_00 This patient's bitemporal hair thinning and large pelvic mass are likely due to a Sertoli-Leydig cell tumor, an ovarian testosterone-secreting sex cord-stromal tumor. The Sertoli and Leydig cells are normally found in the male testes but can develop in the ovaries and produce testosterone. With the malignant proliferation of these cells, male-range testosterone levels can occur and result in rapid-onset bitemporal hair thinning and other signs of virilization (eg, voice deepening, increased muscle mass, clitoromegaly). Because the elevated testosterone levels suppress pituitary FSH and LH release, patients may also develop signs of anovulation and estrogen deficiency such as abnormal uterine bleeding and amenorrhea. Even with a large mass, Sertoli-Leydig cell tumors are typically at an early cancer stage and treatment is with surgical removal. (Choice A) Choriocarcinoma is a malignant gestational trophoblastic neoplasia that typically follows a pregnancy (eg, hydatidiform mole) and may present with abnormal uterine bleeding. However, choriocarcinomas secrete markedly elevated hCG levels (>100,000 mIU/mL), resulting in a positive pregnancy test (not seen in this patient). (Choice B) Ovarian thecomas are benign sex cord-stromal tumors that typically occur in postmenopausal women. Thecomas may secrete estrogen and cause abnormal uterine/postmenopausal bleeding; they do not cause virilization. (Choice C) Polycystic ovary syndrome causes hirsutism and abnormal uterine bleeding due to ovarian hyperandrogenism and chronic anovulation. However, patients have slowly progressive symptoms (eg, over years) and bilateral, slightly enlarged ovaries rather than a large unilateral mass. (Choice E) Struma ovarii is a type of teratoma (germ cell tumor) composed of mature thyroid tissue. Patients can have amenorrhea and a pelvic mass; however, they have signs of hyperthyroidism (eg, tachycardia, weight loss) rather than virilization. (Choice F) Uterine leiomyomas can appear as large pelvic masses with bulk symptoms (eg, abdominal bloating); however, patients typically have regular, heavy menses rather than amenorrhea. Leiomyomas do not cause virilization. Educational objective: Sertoli-Leydig cell tumors are testosterone-secreting sex cord-stromal tumors that can present with rapid-onset virilization (eg, bitemporal hair thinning), amenorrhea, and a large pelvic mass. References: Ovarian sex cord-stromal tumours and their mimics. (http://www.ncbi.nlm.nih.gov/pubmed/29132723) Sertoli-leydig cell tumor of ovary - a diagnostic dilemma. (http://www.ncbi.nlm.nih.gov/pubmed/24783104)

A 38-Year-Old Woman, Gravida 3 Para 3, Comes to the Office