Evaluation of hirsutism
- Overview
- Theory
- Emergencies
- Diagnosis
- Resources
Differentials
Common
Polycystic ovary syndrome
History
oligomenorrhea (<8 periods a year), infertility
Exam
clinical signs of hyperandrogenism other than hirsutism (acne, male-pattern alopecia); obesity (in about 50% of women); acanthosis nigricans may be present
1st investigation
- transvaginal ultrasound:
≥12 follicles measuring 2 mm to 9 mm in diameter and/or increased ovarian volume (>10 mL) in follicular phase ovary[21]Zawadski JK, Dunaif A. Diagnostic criteria for polycystic ovary syndrome: towards a rational approach. In: Dunaif A, Givens JR, Haseltine FP, et al., eds. Polycystic ovary syndrome. Boston: Blackwell Scientific Publications; 1992:377-81.
More transvaginal ultrasoundUltrasound is not recommended in adolescents, as there are no definitive criteria to define polycystic ovary morphology in this population.[11]Teede HJ, Tay CT, Laven J, et al. Recommendations from the 2023 international evidence-based guideline for the assessment and management of polycystic ovary syndrome. Fertil Steril. 2023 Oct;120(4):767-93. https://www.fertstert.org/article/S0015-0282(23)00719-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/37589624?tool=bestpractice.com However, it may be considered If serum androgen levels or the degree of virilization is concerning for an ovarian tumor.[22]. Screening and Management of the Hyperandrogenic Adolescent: ACOG Committee Opinion, Number 789. Obstet Gynecol. 2019 Oct;134(4):e106-e114. https://www.doi.org/10.1097/AOG.0000000000003475 http://www.ncbi.nlm.nih.gov/pubmed/31568365?tool=bestpractice.com
Other investigations
- total testosterone:
elevated
More total testosteroneThe best method for evaluation of total testosterone is liquid chromatography with tandem mass spectrometry (LC-MS/MS).[11]Teede HJ, Tay CT, Laven J, et al. Recommendations from the 2023 international evidence-based guideline for the assessment and management of polycystic ovary syndrome. Fertil Steril. 2023 Oct;120(4):767-93. https://www.fertstert.org/article/S0015-0282(23)00719-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/37589624?tool=bestpractice.com This has higher specificity and less interference with other androgens, such as androstenedione and dehydroepiandrosterone sulfate (DHEAS).[14]Barth JH, Field HP, Yasmin E, et al. Defining hyperandrogenism in polycystic ovary syndrome: measurement of testosterone and androstenedione by liquid chromatography-tandem mass spectrometry and analysis by receiver operator characteristic plots. Eur J Endocrinol. 2010 Mar;162(3):611-5. http://www.ncbi.nlm.nih.gov/pubmed/20008514?tool=bestpractice.com [23]Sairish A, Mudasar N, Shayaq ul Abeer R et al. Hyperandrogenism in polycystic ovarian syndrome and role of CYP gene variants: a review. Egypt. J. Med. Hum.Genet. 2019 Nov 20;20(25). https://jmhg.springeropen.com/articles/10.1186/s43042-019-0031-4 Endocrine Society guidelines recommend that early morning serum total and free testosterone are measured by calculating free androgen index in premenopausal women who have normal total testosterone but who have moderate to severe sexual hair growth or mild sexual hair growth in the presence of symptoms or signs of a hyperandrogenic endocrine disorder, such as menstrual disturbance.[6]Martin KA, Anderson RR, Chang RJ, et al. Evaluation and treatment of hirsutism in premenopausal women: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 Apr 1;103(4):1233-57. https://academic.oup.com/jcem/article/103/4/1233/4924418 http://www.ncbi.nlm.nih.gov/pubmed/29522147?tool=bestpractice.com
Elevated total testosterone concentrations >2 times the upper limit of normal or a history of rapid virilization are likely to be associated with tumor-associated hyperandrogenism. In such cases dehydroepiandrosterone sulfate and androstenedione should be measured to identify an adrenal or ovarian source of the hyperandrogenemia.[16]Sathyapalan, T, Atkin SL. Rational testing: Investigating hirsutism. BMJ. 2009 Apr 28;338:b912. http://www.ncbi.nlm.nih.gov/pubmed/19401328?tool=bestpractice.com
Idiopathic hirsutism
History
regular menstrual cycles, no identifiable etiology for the excessive hair growth
Exam
mild to moderate hirsutism
1st investigation
- total testosterone:
normal
More total testosteroneThe best method for evaluation of total testosterone is liquid chromatography with tandem mass spectrometry (LC-MS/MS). This has higher specificity and less interference with other androgens, such as androstenedione and dehydroepiandrosterone sulfate (DHEAS).[14]Barth JH, Field HP, Yasmin E, et al. Defining hyperandrogenism in polycystic ovary syndrome: measurement of testosterone and androstenedione by liquid chromatography-tandem mass spectrometry and analysis by receiver operator characteristic plots. Eur J Endocrinol. 2010 Mar;162(3):611-5. http://www.ncbi.nlm.nih.gov/pubmed/20008514?tool=bestpractice.com [23]Sairish A, Mudasar N, Shayaq ul Abeer R et al. Hyperandrogenism in polycystic ovarian syndrome and role of CYP gene variants: a review. Egypt. J. Med. Hum.Genet. 2019 Nov 20;20(25). https://jmhg.springeropen.com/articles/10.1186/s43042-019-0031-4 If total testosterone is normal, free testosterone can be assessed by measurement of serum total testosterone and sex hormone binding protein, followed by calculation of the free androgen index.[24]Vermeulen A, Verdonck L, Kaufman JM. A critical evaluation of simple methods for the estimation of free testosterone in serum. J Clin Endocrinol Metab. 1999 Oct;84(10):3666-72. https://www.doi.org/10.1210/jcem.84.10.6079 http://www.ncbi.nlm.nih.gov/pubmed/10523012?tool=bestpractice.com
Endocrine Society guidelines recommend measuring early morning serum total and free testosterone in premenopausal women who have normal total testosterone, but who have moderate to severe sexual hair growth or mild sexual hair growth in the presence of symptoms or signs suggesting a hyperandrogenic endocrine disorder, such as menstrual disturbance.[6]Martin KA, Anderson RR, Chang RJ, et al. Evaluation and treatment of hirsutism in premenopausal women: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 Apr 1;103(4):1233-57. https://academic.oup.com/jcem/article/103/4/1233/4924418 http://www.ncbi.nlm.nih.gov/pubmed/29522147?tool=bestpractice.com
Other investigations
Uncommon
Hyperprolactinemia
History
oligomenorrhea, galactorrhea, infertility
Exam
galactorrhea, may have bilateral hemianopsia
1st investigation
Other investigations
- MRI of hypothalamic/pituitary area:
may show sellar, or suprasellar mass
More MRI of hypothalamic/pituitary areaCT may be an alternative, but MRI is more sensitive for small lesions.[25]Schlechte JA. Clinical practice. Prolactinoma. N Engl J Med. 2003 Nov 20;349(21):2035-41. http://www.ncbi.nlm.nih.gov/pubmed/14627789?tool=bestpractice.com
Nonclassic congenital adrenal hyperplasia
History
premature pubarche, short stature, acne, menstrual irregularity, oligomenorrhea, primary amenorrhea, infertility, early onset of hirsutism, family history of congenital adrenal hyperplasia[26]Azziz R, Sanchez LA, Knochenhauer ES, et al. Androgen excess in women: experience with over 1000 consecutive patients. J Clin Endocrinol Metab. 2004 Feb;89(2):453-62. https://www.doi.org/10.1210/jc.2003-031122 http://www.ncbi.nlm.nih.gov/pubmed/14764747?tool=bestpractice.com
Exam
acne, clitoromegaly (10% of the patients), alopecia (8%)[26]Azziz R, Sanchez LA, Knochenhauer ES, et al. Androgen excess in women: experience with over 1000 consecutive patients. J Clin Endocrinol Metab. 2004 Feb;89(2):453-62. https://www.doi.org/10.1210/jc.2003-031122 http://www.ncbi.nlm.nih.gov/pubmed/14764747?tool=bestpractice.com
1st investigation
- serum 17-hydroxyprogesterone:
>200 nanograms/dL
More serum 17-hydroxyprogesteroneShould be measured in the morning, during follicular phase; levels may be higher in the afternoon. For women with amenorrhea/infrequent menses it can be done on a random day. In women considered to have a high risk of nonclassic congenital adrenal hyperplasia this screening test is recommended even if serum total and free testosterone are normal.[6]Martin KA, Anderson RR, Chang RJ, et al. Evaluation and treatment of hirsutism in premenopausal women: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 Apr 1;103(4):1233-57. https://academic.oup.com/jcem/article/103/4/1233/4924418 http://www.ncbi.nlm.nih.gov/pubmed/29522147?tool=bestpractice.com
Diagnosis should be confirmed by ACTH stimulation test.[27]White PC, Speiser PW. Congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Endocr Rev. 2000 Jun;21(3):245-91. https://www.doi.org/10.1210/edrv.21.3.0398 http://www.ncbi.nlm.nih.gov/pubmed/10857554?tool=bestpractice.com
Other investigations
- adrenocorticotropic hormone (ACTH) stimulation test:
17-hydroxyprogesterone >1500 nanograms/dL
More adrenocorticotropic hormone (ACTH) stimulation testCosyntropin, a synthetic form of ACTH, is injected (0.25 micrograms intravenously/intramuscularly), and measurements of 17-hydroxyprogesterone are done before administration and after 30 and 60 minutes. The diagnosis of nonclassic congenital adrenal hyperplasia is confirmed when levels are >1500 nanograms/dL.[18]Azziz R, Dewailly D, Owerbach D. Clinical review 56: nonclassic adrenal hyperplasia: current concepts. J Clin Endocrinol Metab. 1994 Apr;78(4):810-5. http://www.ncbi.nlm.nih.gov/pubmed/8157702?tool=bestpractice.com
An intramuscular depot formulation of cosyntropin is used in cases of suspected adrenocortical insufficiency where the original tests yield inconclusive results.
Cushing syndrome (benign)
History
menstrual irregularity, bruising, emotional lability
Exam
hypertension, truncal obesity associated with nuchal fat pad, moon face, facial plethora, purple striae, proximal muscle weakness
1st investigation
- salivary cortisol:
elevated
More salivary cortisolOne of the first-line tests to consider in any patient with suspected Cushing syndrome.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com
Collected at 11 p.m.
Obtaining multiple (at least two) samples may increase sensitivity, and initial testing should be performed with sampling on two separate nights.[29]Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008 May;93(5):1526-40. http://www.ncbi.nlm.nih.gov/pubmed/18334580?tool=bestpractice.com [30]Carroll T, Raff H, Findling JW. Late-night salivary cortisol for the diagnosis of Cushing syndrome: a meta-analysis. Endocr Pract. 2009 May-Jun;15(4):335-42. https://www.endocrinepractice.org/article/S1530-891X(20)41041-9/fulltext http://www.ncbi.nlm.nih.gov/pubmed/19502211?tool=bestpractice.com [31]Raff H. Utility of salivary cortisol measurements in Cushing's syndrome and adrenal insufficiency. J Clin Endocrinol Metab. 2009 Oct;94(10):3647-55. https://academic.oup.com/jcem/article/94/10/3647/2596462 http://www.ncbi.nlm.nih.gov/pubmed/19602555?tool=bestpractice.com [32]Zhang Q, Dou J, Gu W, et al. Reassessing the reliability of the salivary cortisol assay for the diagnosis of Cushing syndrome. J Int Med Res. 2013 Oct;41(5):1387-94. https://journals.sagepub.com/doi/10.1177/0300060513498017 http://www.ncbi.nlm.nih.gov/pubmed/24065452?tool=bestpractice.com Value greater than the upper limit of normal is considered positive. Normal values vary greatly depending on the assay and clinical imaging used. Positive results should be confirmed with dexamethasone suppression testing or 24-hour urinary free cortisol.
- 24-hour urinary free cortisol:
elevated
More 24-hour urinary free cortisolAssay-specific reference range.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com Variability may be 50% between samples, thus 2 to 3 collections are required.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com
Other investigations
- serum cortisol post overnight dexamethasone suppression test:
1.8 micrograms/dL (>50 nanomol/L)
More serum cortisol post overnight dexamethasone suppression test1 mg of dexamethasone given at 11 p.m. and serum cortisol collected at 8 a.m. the next morning.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com [29]Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008 May;93(5):1526-40. http://www.ncbi.nlm.nih.gov/pubmed/18334580?tool=bestpractice.com
Androgenic medications
History
use of androgenic medications (anabolic or androgenic steroids, danazol, oral contraceptives with androgenic progestins)
Exam
mild to moderate hirsutism
1st investigation
- stop medication:
improvement in hirsutism
Other investigations
Androgen-secreting ovarian tumor
History
older age, rapid progression, virilization
Exam
signs of virilization; palpable abdominal mass or palpable mass on vaginal examination
1st investigation
- total testosterone:
elevated
More total testosteroneThe best method for evaluation of total testosterone is liquid chromatography with tandem mass spectrometry (LC-MS/MS). This has higher specificity and less interference with other androgens, such as androstenedione and dehydroepiandrosterone sulfate (DHEAS).[14]Barth JH, Field HP, Yasmin E, et al. Defining hyperandrogenism in polycystic ovary syndrome: measurement of testosterone and androstenedione by liquid chromatography-tandem mass spectrometry and analysis by receiver operator characteristic plots. Eur J Endocrinol. 2010 Mar;162(3):611-5. http://www.ncbi.nlm.nih.gov/pubmed/20008514?tool=bestpractice.com [23]Sairish A, Mudasar N, Shayaq ul Abeer R et al. Hyperandrogenism in polycystic ovarian syndrome and role of CYP gene variants: a review. Egypt. J. Med. Hum.Genet. 2019 Nov 20;20(25). https://jmhg.springeropen.com/articles/10.1186/s43042-019-0031-4 If total testosterone is normal, free testosterone can be assessed by measurement of serum total testosterone and sex hormone binding protein, followed by calculation of the free androgen index.[24]Vermeulen A, Verdonck L, Kaufman JM. A critical evaluation of simple methods for the estimation of free testosterone in serum. J Clin Endocrinol Metab. 1999 Oct;84(10):3666-72. https://www.doi.org/10.1210/jcem.84.10.6079 http://www.ncbi.nlm.nih.gov/pubmed/10523012?tool=bestpractice.com
Endocrine Society guidelines recommend measuring early morning serum total and free testosterone in premenopausal women who have normal total testosterone, but who have moderate to severe sexual hair growth or mild sexual hair growth in the presence of symptoms or signs suggesting a hyperandrogenic endocrine disorder, such as menstrual disturbance.[6]Martin KA, Anderson RR, Chang RJ, et al. Evaluation and treatment of hirsutism in premenopausal women: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 Apr 1;103(4):1233-57. https://academic.oup.com/jcem/article/103/4/1233/4924418 http://www.ncbi.nlm.nih.gov/pubmed/29522147?tool=bestpractice.com
Elevated total testosterone concentrations >2 times the upper limit of normal or a history of rapid virilization are likely to be associated with tumor-associated hyperandrogenism. In such cases, dehydroepiandrosterone sulfate and androstenedione should be measured to identify an adrenal or ovarian source of the hyperandrogenemia.[16]Sathyapalan, T, Atkin SL. Rational testing: Investigating hirsutism. BMJ. 2009 Apr 28;338:b912. http://www.ncbi.nlm.nih.gov/pubmed/19401328?tool=bestpractice.com
- pelvic ultrasound:
adnexal mass
Other investigations
- androstenedione:
elevated
Ovarian hyperthecosis
History
may occur after menopause, intense hirsutism of slow progression, virilization, amenorrhea, or irregular menses
Exam
obesity, virilization (clitoromegaly, temporal balding, deepening of the voice, increased muscle mass), moderate to severe hirsutism, acanthosis nigricans
1st investigation
- total testosterone:
elevated
More total testosteroneThe best method for evaluation of total testosterone is liquid chromatography with tandem mass spectrometry (LC-MS/MS). This has higher specificity and less interference with other androgens, such as androstenedione and dehydroepiandrosterone sulfate (DHEAS).[14]Barth JH, Field HP, Yasmin E, et al. Defining hyperandrogenism in polycystic ovary syndrome: measurement of testosterone and androstenedione by liquid chromatography-tandem mass spectrometry and analysis by receiver operator characteristic plots. Eur J Endocrinol. 2010 Mar;162(3):611-5. http://www.ncbi.nlm.nih.gov/pubmed/20008514?tool=bestpractice.com [23]Sairish A, Mudasar N, Shayaq ul Abeer R et al. Hyperandrogenism in polycystic ovarian syndrome and role of CYP gene variants: a review. Egypt. J. Med. Hum.Genet. 2019 Nov 20;20(25). https://jmhg.springeropen.com/articles/10.1186/s43042-019-0031-4 If total testosterone is normal, free testosterone can be assessed by measurement of serum total testosterone and sex hormone binding protein, followed by calculation of the free androgen index.[24]Vermeulen A, Verdonck L, Kaufman JM. A critical evaluation of simple methods for the estimation of free testosterone in serum. J Clin Endocrinol Metab. 1999 Oct;84(10):3666-72. https://www.doi.org/10.1210/jcem.84.10.6079 http://www.ncbi.nlm.nih.gov/pubmed/10523012?tool=bestpractice.com
Endocrine Society guidelines recommend measuring early morning serum total and free testosterone in premenopausal women who have normal total testosterone, but who have moderate to severe sexual hair growth or mild sexual hair growth in the presence of symptoms or signs suggesting a hyperandrogenic endocrine disorder, such as menstrual disturbance.[6]Martin KA, Anderson RR, Chang RJ, et al. Evaluation and treatment of hirsutism in premenopausal women: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2018 Apr 1;103(4):1233-57. https://academic.oup.com/jcem/article/103/4/1233/4924418 http://www.ncbi.nlm.nih.gov/pubmed/29522147?tool=bestpractice.com
- transvaginal ultrasound:
increase in ovarian stroma with hyperechogenicity and few or absent cysts
More transvaginal ultrasoundOvaries may be very small and not visible. In menopausal women, may be discovered only after oophorectomy.
Other investigations
Adrenocortical carcinoma
History
older age, rapidly progressive hirsutism, virilization
Exam
hypertension, virilization, cushingoid features (bruising, thin skin, facial plethora, purple striae)
1st investigation
- salivary cortisol:
elevated
More salivary cortisolCollected at 11 p.m.
Obtaining multiple (at least two) samples may increase sensitivity, and initial testing should be performed with sampling on two separate nights.[29]Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008 May;93(5):1526-40. http://www.ncbi.nlm.nih.gov/pubmed/18334580?tool=bestpractice.com [30]Carroll T, Raff H, Findling JW. Late-night salivary cortisol for the diagnosis of Cushing syndrome: a meta-analysis. Endocr Pract. 2009 May-Jun;15(4):335-42. https://www.endocrinepractice.org/article/S1530-891X(20)41041-9/fulltext http://www.ncbi.nlm.nih.gov/pubmed/19502211?tool=bestpractice.com [31]Raff H. Utility of salivary cortisol measurements in Cushing's syndrome and adrenal insufficiency. J Clin Endocrinol Metab. 2009 Oct;94(10):3647-55. https://academic.oup.com/jcem/article/94/10/3647/2596462 http://www.ncbi.nlm.nih.gov/pubmed/19602555?tool=bestpractice.com [32]Zhang Q, Dou J, Gu W, et al. Reassessing the reliability of the salivary cortisol assay for the diagnosis of Cushing syndrome. J Int Med Res. 2013 Oct;41(5):1387-94. https://journals.sagepub.com/doi/10.1177/0300060513498017 http://www.ncbi.nlm.nih.gov/pubmed/24065452?tool=bestpractice.com Value greater than the upper limit of normal is considered positive.
Normal values vary greatly depending on the assay and clinical imaging used.
Positive results should be confirmed with dexamethasone suppression testing or 24-hour urinary free cortisol.
- 24-hour urinary free cortisol:
elevated
More 24-hour urinary free cortisolAssay-specific reference range.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com Variability may be 50% between samples, thus 2 to 3 collections are required.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com
- total testosterone:
elevated
More total testosteroneThe best method for evaluation of total testosterone is liquid chromatography with tandem mass spectrometry (LC-MS/MS).[11]Teede HJ, Tay CT, Laven J, et al. Recommendations from the 2023 international evidence-based guideline for the assessment and management of polycystic ovary syndrome. Fertil Steril. 2023 Oct;120(4):767-93. https://www.fertstert.org/article/S0015-0282(23)00719-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/37589624?tool=bestpractice.com This has higher specificity and less interference with other androgens, such as androstenedione and dehydroepiandrosterone sulfate (DHEAS).[14]Barth JH, Field HP, Yasmin E, et al. Defining hyperandrogenism in polycystic ovary syndrome: measurement of testosterone and androstenedione by liquid chromatography-tandem mass spectrometry and analysis by receiver operator characteristic plots. Eur J Endocrinol. 2010 Mar;162(3):611-5. http://www.ncbi.nlm.nih.gov/pubmed/20008514?tool=bestpractice.com [23]Sairish A, Mudasar N, Shayaq ul Abeer R et al. Hyperandrogenism in polycystic ovarian syndrome and role of CYP gene variants: a review. Egypt. J. Med. Hum.Genet. 2019 Nov 20;20(25). https://jmhg.springeropen.com/articles/10.1186/s43042-019-0031-4 Elevated total testosterone concentrations >2 times the upper limit of normal or a history of rapid virilization are likely to be associated with tumor-associated hyperandrogenism. In such cases, dehydroepiandrosterone sulfate and androstenedione should be measured to identify an adrenal or ovarian source of the hyperandrogenemia.
Other investigations
- serum cortisol post overnight dexamethasone suppression test:
>50 nanomol/L (1.8 micrograms/dL)
More serum cortisol post overnight dexamethasone suppression test1 mg of dexamethasone given at 11 p.m. and serum cortisol collected at 8 a.m. the next morning.[28]Fleseriu M, Auchus R, Bancos I, et al. Consensus on diagnosis and management of Cushing's disease: a guideline update. Lancet Diabetes Endocrinol. 2021 Dec;9(12):847-75. https://www.thelancet.com/journals/landia/article/PIIS2213-8587(21)00235-7/fulltext http://www.ncbi.nlm.nih.gov/pubmed/34687601?tool=bestpractice.com [29]Nieman LK, Biller BM, Findling JW, et al. The diagnosis of Cushing's syndrome: an Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2008 May;93(5):1526-40. http://www.ncbi.nlm.nih.gov/pubmed/18334580?tool=bestpractice.com
Cortisol-secreting adrenocortical carcinoma is associated with poor outcome.[33]Vanbrabant T, Fassnacht M, Assie G, et al. Influence of hormonal functional status on survival in adrenocortical carcinoma: systematic review and meta-analysis. Eur J Endocrinol. 2018 Dec 1;179(6):429-36. https://academic.oup.com/ejendo/article-abstract/179/6/429/6655440?redirectedFrom=fulltext http://www.ncbi.nlm.nih.gov/pubmed/30325179?tool=bestpractice.com
- dehydroepiandrosterone sulfate:
elevated
- CT or MRI of the adrenals:
features suggestive of malignancy, such as size >6 cm, unenhanced CT attenuation >10 Hounsfield units, invasion of surrounding structures, and irregular borders
More CT or MRI of the adrenalsMay be helpful in distinguishing adenomas from carcinomas.[19]Ng L, Libertino JM. Adrenocortical carcinoma: diagnosis, evaluation and treatment. J Urol. 2003 Jan;169(1):5-11. http://www.ncbi.nlm.nih.gov/pubmed/12478091?tool=bestpractice.com [20]Fassnacht M, Kenn W, Allolio B. Adrenal tumors: how to establish malignancy? J Endocrinol Invest. 2004 Apr;27(4):387-99. http://www.ncbi.nlm.nih.gov/pubmed/15233562?tool=bestpractice.com
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