An Adolescent Female WhoHas Increasing Hair Growth
Tamar Stricker, MD,* Francesca Navratil, MD,*
Felix H. Sennhauser, MD*
PresentationA 14-year-old girl presents to the clinic complaining ofincreasing hair growth on her face and trunk for the past2 years. Her parents note that her normally low-pitchedvoice has seemed even lower the past few months. Breastand pubic hair development began at 11 years of age, andmenstrual periods began at approximately 12 years ofage. Menstrual periods occur every 4 to 8 weeks. The girlis in good health, has never had any serious illness, anddenies taking any medications. She has always been thetallest in her class.
Physical examination reveals a well-nourished femalewho has a deep voice, receding frontal hairline, andconspicuous, dark hair over the upper lip and chin (Fig.1). Minimal facial acne is noted. Vital signs include atemperature of 98°F (36.7°C), respiratory rate of 22breaths/min, pulse of 90 beats/min, and a blood pres-sure of 130/70 mm Hg. Height is 166.9 cm (50th to75th percentile) and weight is 67.7 kg (90th to 97thpercentile). Breast examination reveals elevation of theareola and nipple (Sexual Maturity Rating stage 4), withprominent dark hairs over the areola (Fig. 2). Truncalexamination demonstrates midline hair over the chestand upper abdomen as well as a suggestion of increasedmuscle mass and loss of female contour (Fig. 3). Genito-urinary inspection is remarkable for an enlarged clitoris,measuring 2.5 cm in length. Findings on the remainderof the physical examination are normal.
Serum testosterone and androstenedione levels areapproximately five-fold higher than normal for her age.The serum dehydroepiandrosterone sulfate (DHEAS)concentration is normal. Serum progesterone and estra-diol levels are normal. Serum follicle stimulating hor-mone (FSH) concentration is low, and leuteinizing hor-mone (LH) concentration is normal. Human chorionicgonadotropin (hCG) measurement is normal.
Computed tomography (CT) of the pelvis suggeststhe diagnosis.*University Children’s Hospital, Zurich, Switzerland.
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Diagnosis: Ovarian Tumor (Sertoli-LeydigCell Tumor)CT examination demonstrates a 6-cm long tumor ofmixed solid and cystic appearance in the right ovary (Fig.4). Serum alpha-fetoprotein (AFP) level is elevated.A right oophorectomy reveals a Sertoli-Leydig tumor ofintermediate differentiation confined within the ovary.Six months after surgery, the patient’s hirsutism is re-duced markedly, and her menstrual periods occur inregular, monthly cycles (Fig. 5).
DefinitionsHirsutism and virilization are terms frequently used todescribe excess hair growth in women. Normally, distri-bution of hair depends on age and gender. Fine, lightlypigmented hairs, known as vellus hair, cover most ofthe body. Coarse, darker pigmented hairs, known asterminal hair, grow in the eyebrows, eyelashes, and scalp.During puberty, under the influence of androgens, ter-minal hair replaces vellus hair in the axillae, lower pubictriangle, arms, and lower legs of both genders. In adultmen, terminal hair also develops in the androgen-sensitive regions of the upper lip, chin, sideburns, upperarms, chest (periareolar area and linea alba), upper pubictriangle, thighs, and intergluteal region. In adult women,androgen excess or increased androgen sensitivity maylead to a similar hair distribution.
Hirsutism in women is defined as excess body hair inmale-pattern distribution. Virilization is the concurrentpresentation of hirsutism with other signs of androgenexcess, including frontotemporal alopecia (male-patternbalding), deepening of the voice (laryngeal hypertro-phy), a decrease in breast size, clitoral hypertrophy,
increased muscle mass, and amenorrhea or oligomenor-rhea. Acne often accompanies androgen excess. Hirsut-ism may be the first manifestation of a condition that maylead to virilization. (Of note, hypertrichosis describes anexcess of vellus hair.)
Androgens affect the pilosebaceous unit, leading toincreased activity of the hair follicle and sebaceous gland(development of terminal hair and acne). Serum andro-gens include testosterone, free testosterone, dehydroepi-androsterone (DHEA), DHEAS, and androstenedione.Testosterone is converted to dihydrotestosterone(DHT) in the skin and the hair follicle by 5-alpha-reductase. In women, approximately 50% of circulatingandrogens arise from the ovaries and adrenals, with theovary as a source of testosterone, androstenedione, andDHEA and the adrenal as a source of androstenedione,DHEA, and DHEAS. Androstenedione from the adrenalis converted peripherally to testosterone. Ovarian andro-gen production is gonadotrophin-dependent; adrenalandrogen production is adrenocorticotropic hormone
Figure 4. Pelvic CT demonstrating a cystic solid mass in theright ovary. (Courtesy of Ulrich V. Willi, MD) Figure 5. Six months after oophorectomy.
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(ACTH)-dependent. Most of the circulating testoster-one and DHT is bound to sex hormone-binding globu-lin (SHBG). A decrease in SHBG levels leads to a relativeincrease in free testosterone.
EtiologyHirsutism can result from excess androgen production,relative circulating androgen excess due to low levels ofbinding globulins, excess end-organ response, or patientperception. The evaluation should assess whether hirsut-ism is accompanied by associated pilosebaceous unitoveractivity, ovulation disorder, or signs of virilization.In young women, most cases of virilization are acute andresult from increased adrenal or ovarian androgen pro-duction, a decrease in SHBG, or the use of exogenousandrogens.
Differential DiagnosisFor disorders of excess androgen, the differential diag-nosis includes exogenous androgens, disorders of theovary or adrenal, and disorders of sexual differentiation(mixed gonadal dysgenesis). Disorders of the ovary in-clude polycystic ovary syndrome (PCOS), severe insulinresistance, hyperthecosis, tumors, and enzyme defi-ciency. Features of PCOS, the most common cause ofacute hirsutism in adolescent females, include hirsutismwith obesity, insulin resistance, acanthosis nigricans (vel-vety, thickened hyperpigmentation of the axillae, neck,anogenital area, and groin), oligomenorrhea, anovula-tion, and infertility. Disorders of the adrenal includelate-onset congenital adrenal hyperplasia, Cushing syn-drome, and tumors.
Drugs (anabolic steroids, minoxidil, acetazolamide,danazol, valproate, glucocorticoids), pregnancy, an-orexia nervosa, hypothyroidism, and malnutrition may
cause relative circulating androgen excess due to lowbinding globulins.
Genetic, idiopathic, familial, or racial factors maycause excess end-organ response to androgens.
ExaminationThe history and physical examination help determine thecause of hirsutism. It is important to note the patient’sethnic background; body perception; menstrual history;drug history; and the age of onset, progression, andduration of hirsutism. The physical examination shouldinclude evaluation of height and weight, blood pressure,body habitus, and pubertal development. Past photo-graphs may help assess changes in body habitus. Use ofsubjective clinical scoring systems, such as the FerimanGallwey scale, help measure the degree of conversion ofvellus to terminal hair. An abdominal examination mayreveal a palpable mass. Other physical signs suggestive ofandrogen excess are acne, acanthosis nigricans, galactor-rhea, central obesity, and striae.
Laboratory evaluation is warranted if physical exami-nation reveals virilization and the presence of exogenousandrogens is ruled out. Elevated serum total and freetestosterone levels may reveal endogenous androgen ex-cess from the ovary or adrenal. An elevated serumDHEAS level indicates adrenal pathology. An elevatedfasting 17-hydroxyprogesterone level may indicate con-genital adrenal hyperplasia (CAH). Often, an ACTHstimulation test is required to diagnose late-onset CAH.Abnormal FSH or LH levels may reveal an ovulationdisorder; a high LH concentration is suggestive ofPCOS. Normal gonadotropin levels do not, however,rule out PCOS. Suppressed gonadotropins, as in thiscase, strongly suggest either exogenous administrationor autonomous overproduction of androgens from atumor. Pelvic imaging studies, including ultrasonogra-phy, CT, or magnetic resonance imaging, may reveal anunderlying tumor.
TreatmentTreatment of isolated hirsutism in an adolescent focuseson preventing further stimulation of hair growth bydecreasing androgen levels (eg, metformin administra-tion), increasing SHBG (eg, estrogen administration), orblocking androgen action (eg, spironolactone adminis-tration). Cosmetic correction of already stimulated hairoften is necessary. Virilization in an adolescent frequentlyindicates a malignant process that requires surgery.
Sertoli-Leydig cell tumor of the ovary, previouslytermed androblastoma or arrhenoblastoma, is a sex cord-
Definitions
Feriman Gallwey score—A semiquantitative scorebased on the distribution of hair on 11 body sites.Used for the clinical assessment of hirsutism, it alsomay help to monitor treatment outcome.Hirsutism—Excessive male-pattern hair growth inwomen.Virilization—Concurrent presentation of hirsutismwith other signs of androgen excess, such as acne,frontotemporal balding, deepening of the voice,clitoral hypertrophy, and increased muscle mass.
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stromal tumor that accounts for 0.5% of all ovariantumors. Ovarian Sertoli-Leydig cell tumor is classifiedinto five histologic categories: well-differentiated, inter-mediately differentiated, poorly differentiated, retiform,and mixed. Younger patients tend to have more poorlydifferentiated tumors. Age at presentation ranges from7 to 79 years, with the average being 25 years. Clinicalsymptoms include amenorrhea, abdominal mass, viriliza-tion, and in young children, heterosexual precocity. Thetumor marker AFP may be elevated. Treatment consistsof surgical excision, chemotherapy, or both, dependingon staging. The prognosis generally is good.
SummaryHirsutism affects the well-being of adolescent womenand may be the manifestation of a serious underlyingdisorder. Virilization, if present, requires a prompt, thor-ough, and comprehensive diagnostic evaluation.
Comment: Although ovarian tumors are rare in ado-lescent females, PCOS is not. This case provides a good
overview of the presentation and evaluation of hirsutismand its infrequent companion, virilization.
Joseph ZenelEditor, Visual Diagnosis
Suggested ReadingBorer JG, Tan PE, Diamond DA. The spectrum of Sertoli cell
tumors in children. Urol Clin North Am. 2000;27:529–541Conn JJ, Jacobs HS. The clinical management of hirsutism. Eur J
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