Non-PCOS Hyperandrogenic Disorders in Adolescents

Abstract

Hyperandrogenism—clinical features resulting from increased androgen production and/or action—is not uncommon in peripubertal girls. Hyperandrogenism affects 3 to 20% of adolescent girls and often is associated with hyperandrogenemia. In prepubertal girls, the most common etiologies of androgen excess are premature adrenarche (60%) and congenital adrenal hyperplasia (CAH; 4%). In pubertal girls, polycystic ovary syndrome (PCOS; 20–40%) and CAH (14%) are the most common diagnoses related to androgen excess. Androgen-secreting ovarian or adrenal tumors are rare (0.2%). Early pubic hair, acne, and/or hirsutism are the most common clinical manifestations, but signs of overt virilization in adolescent girls—rapid progression of pubic hair or hirsutism, clitoromegaly, voice deepening, severe cystic acne, growth acceleration, increased muscle mass, and bone age advancement past height age—should prompt detailed evaluation. This article addresses the clinical manifestations of and management considerations for non-PCOS-related hyperandrogenism in adolescent girls. We propose an algorithm to aid diagnostic evaluation of androgen excess in this specific patient population.

Fifteen-minute consultation: Approach to the adolescent presenting with hirsutism

“…Idiopathic hirsutism, with or without hyperandrogenism, is common, but the the most common cause in adolescents is polycystic ovarian syndrome (PCOS) 3 4. The diagnosis of PCOS is challenging in adolescents due to a considerable overlap with the healthy population as features like acne and irregular menstrual cycles are commonplace.…”

“…Rarer conditions, including non-classic congenital adrenal hyperplasia (NCCAH), androgen-secreting tumours and other endocrinopathies may present with hirsutism and are important to consider (table 1). 3…”

“…It is important to explore the patient’s (and parent’s/carer’s) expectations to aid empathic discussion of realistic and achievable treatment outcomes. Detailed history should be sought about the age/stage of puberty at onset and speed of hair growth (prepubertal onset and rapid progression are concerning for androgen secreting tumours) 3. Often patients have removed the hair, so a description of the hair and its distribution will help assess severity and differentiate it from hypertrichosis.…”

“…Hirsutism should be differentiated from hypertrichosis, generalised excess hair growth in non-androgen-dependent areas, for example, the forearms 2. Hirsutism is a common condition, estimated to affect 3%–20% of adolescent girls3 with associated psychosocial morbidity 1…”

Criteria for Diagnosis of Polycystic Ovary Syndrome during Adolescence: Literature Review

“…Some blood tests are essential to diagnose PCOS in adolescent and adult women for the exclusion of other disorders that can cause irregular menstrual cycles and/or hyperandrogenism including beta human chorionic gonadotropin hormone (if sexually active), LH, FSH, thyroid function tests, prolactin, midnight salivary cortisol, and 17-hydroxyprogesterone (17-OHP) [ 79 , 80 ]. Demirci et al investigated whether any other indicator could distinguish PCOS from non-classic congenital adrenal hyperplasia.…”