A 16-year-old girl comes to the office for evaluation of acne. Around the time of her first menstrual period at age 10, the acne appeared only on her face, but over the past 6 years, it has spread to her chest and back. Topical preparations are not effective, and the patient feels anxious regarding social situations at school. She quit the soccer team and has since gained 9.1 kg (20 lb). The patient has never been sexually active. Menses are irregular and her last menstrual period was 3 months ago. She has not had a Pap test but has completed the human papillomavirus vaccination series. The patient takes no medications and does not use tobacco. Family history is noncontributory. Physical examination shows cystic and nodular acne over the face, back, and upper chest. There is no goiter or thyroid masses. Terminal hairs are notable on the upper lip and around the nipples. Development is Tanner stage V. The abdomen is obese and nontender, with no masses and normal bowel sounds. Serum total testosterone and dehydroepiandrosterone sulfate levels are normal. This patient is at greatest risk for developing malignancy of which of the following?

Question

A 16-year-old girl comes to the office for evaluation of acne.  Around the time of her first menstrual period at age 10, the acne appeared only on her face, but over the past 6 years, it has spread to her chest and back.  Topical preparations are not effective, and the patient feels anxious regarding social situations at school.  She quit the soccer team and has since gained 9.1 kg (20 lb).  The patient has never been sexually active.  Menses are irregular and her last menstrual period was 3 months ago.  She has not had a Pap test but has completed the human papillomavirus vaccination series.  The patient takes no medications and does not use tobacco.  Family history is noncontributory.  Physical examination shows cystic and nodular acne over the face, back, and upper chest.  There is no goiter or thyroid masses.  Terminal hairs are notable on the upper lip and around the nipples.  Development is Tanner stage V.  The abdomen is obese and nontender, with no masses and normal bowel sounds.  Serum total testosterone and dehydroepiandrosterone sulfate levels are normal.  This patient is at greatest risk for developing malignancy of which of the following?

Quizplus · Accepted Answer

11ec25c8_d9df_273b_bc81_d340fbfc9e6e_MD0003_00 Polycystic ovary syndrome (PCOS) is a common condition diagnosed in adolescents due to signs of hyperandrogenism and irregular menses. Clinical evidence of hyperandrogenism includes severe nodulocystic acne, male pattern baldness, and hirsutism. Although many patients with PCOS have biochemical evidence of hyperandrogenism with elevated serum levels of total testosterone, these laboratory values may be normal in some due to decreased levels of sex hormone-binding globulin, with elevated free testosterone levels instead. Therefore, the diagnosis requires either clinical or biochemical evidence of hyperandrogenism. Women with PCOS typically have decreased progesterone secretion due to chronic anovulatory cycles. Therefore, these patients usually have a constant and unbalanced proliferation of the endometrium by estrogens. This unopposed estrogen stimulation places patients at increased risk for endometrial hyperplasia and cancer. Treatment with cyclic progesterone, estrogen/progestin oral contraceptives, or progesterone-releasing intrauterine devices protects the endometrium from hyperplasia and reduces cancer risk. proliferation of the endometrium by estrogens (Choice A) Adrenocortical malignancies typically occur with an associated syndrome (eg, Li-Fraumeni syndrome). An adrenocortical tumor may cause hyperandrogenism but is unlikely in a patient with a negative family history and normal dehydroepiandrosterone sulfate. (Choices B and F) The main risk factor for squamous cell vulvar and cervical cancer is persistent human papillomavirus (HPV) infection. The HPV vaccination series reduces the risk of these cancers by preventing infection with high-risk HPV types 16 and 18. (Choice D) Family history and radiation exposure are the major risk factors for thyroid cancer. Typical presentation is a thyroid nodule and no menstrual irregularities. (Choice E) Clear cell vaginal cancer is linked to in utero exposure to diethylstilbestrol, not PCOS. Vaginal squamous cell carcinoma is linked to HPV infection. Educational objective: Polycystic ovary syndrome (PCOS) presents with hyperandrogenism (eg, severe acne, hirsutism, androgenic alopecia) and irregular menses. Patients with PCOS are at increased risk for endometrial hyperplasia and cancer due to unregulated endometrial proliferation from unopposed estrogen stimulation. References: ACOG Practice Bulletin No. 108: polycystic ovary syndrome. (http://www.ncbi.nlm.nih.gov/pubmed/19888063) Genetic, hormonal and metabolic aspects of PCOS: an update. (http://www.ncbi.nlm.nih.gov/pubmed/27423183)

A 16-Year-Old Girl Comes to the Office for Evaluation of Acne