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Frontiers in Endocrinology 2023Adult pure androgen-secreting adrenal tumors (PASATs) are extremely rare, and their characteristics are largely unknown.
BACKGROUND
Adult pure androgen-secreting adrenal tumors (PASATs) are extremely rare, and their characteristics are largely unknown.
METHODS
A rare case of adult bilateral PASATs was reported, and a systematic literature review of adult PASATs was conducted to summarize the characteristics of PASATs.
RESULTS
In total, 48 studies, including 40 case reports and 8 articles, were identified in this review. Analysis based on data of 42 patients (including current case and 41 patients from 40 case reports) showed that average age was 40.48 ± 15.80 years (range of 18-76). The incidence of adult PASAT peaked at 21-30 years old, while that of malignant PASAT peaked at 41-50 years old. Most PASAT patients were female (40/42, 95.23%), and hirsutism was the most common symptom (37/39, 94.87%). Testosterone (T) was the most commonly elevated androgen (36/42, 85.71%), and 26 of 32 tested patients presented increased dehydroepiandrosterone sulfate (DS) levels. In malignancy cases, disease duration was significantly decreased (1.96 vs. 4.51 years, P=0.025), and tumor diameter was significantly increased (8.9 vs. 4.9 cm, p=0.011). Moreover, the androgen levels, namely, T/upper normal range limit (UNRL) (11.94 vs. 4.943, P=0.770) and DS/UNRL (16.5 vs. 5.28, P=0.625), were higher in patients with malignancy. In total, 5 out of 7 patients showed an increase in DS or T in the human chorionic gonadotropin (HCG) stimulation test. Overall, 41 out of 42 patients (including current case) underwent adrenal surgery, and recurrence, metastasis, or death was reported in 5 out of 11 malignant patients even with adjuvant or rescue mitotane chemotherapy.
CONCLUSION
Adult PASAT, which is predominant in women, is characterized by virilism and menstrual dysfunction, especially hirsutism. Elevated T and DS may contribute to the diagnosis of adult PASAT, and HCG stimulation test might also be of help in diagnosis. Patients with malignant PASAT have a shorter disease duration, larger tumor sizes and relatively higher androgen levels. Surgery is recommended for all local PASATs, and Malignancy of PASAT should be fully considered due to the high risk of malignancy, poor prognosis and limited effective approaches.
Topics: Adult; Humans; Female; Adolescent; Young Adult; Middle Aged; Aged; Male; Androgens; Hirsutism; Adrenal Gland Neoplasms; Testosterone; Virilism
PubMed: 37033242
DOI: 10.3389/fendo.2023.1138114 -
The Journal of Clinical Endocrinology... Apr 2018To update the "Evaluation and Treatment of Hirsutism in Premenopausal Women: An Endocrine Society Clinical Practice Guideline," published by the Endocrine Society in...
OBJECTIVE
To update the "Evaluation and Treatment of Hirsutism in Premenopausal Women: An Endocrine Society Clinical Practice Guideline," published by the Endocrine Society in 2008.
PARTICIPANTS
The participants include an Endocrine Society-appointed task force of seven medical experts and a methodologist.
EVIDENCE
This evidence-based guideline was developed using the Grading of Recommendations, Assessment, Development, and Evaluation system to describe the strength of recommendations and the quality of evidence. The task force commissioned two systematic reviews and used the best available evidence from other published systematic reviews and individual studies.
CONSENSUS PROCESS
Group meetings, conference calls, and e-mail communications facilitated consensus development. Endocrine Society committees, members, and cosponsoring organizations reviewed and commented on preliminary drafts of the guidelines.
CONCLUSION
We suggest testing for elevated androgen levels in all women with an abnormal hirsutism score. We suggest against testing for elevated androgen levels in eumenorrheic women with unwanted local hair growth (i.e., in the absence of an abnormal hirsutism score). For most women with patient-important hirsutism despite cosmetic measures (shaving, plucking, waxing), we suggest starting with pharmacological therapy and adding direct hair removal methods (electrolysis, photoepilation) for those who desire additional cosmetic benefit. For women with mild hirsutism and no evidence of an endocrine disorder, we suggest either pharmacological therapy or direct hair removal methods. For pharmacological therapy, we suggest oral combined estrogen-progestin contraceptives for the majority of women, adding an antiandrogen after 6 months if the response is suboptimal. We recommend against antiandrogen monotherapy unless adequate contraception is used. We suggest against using insulin-lowering drugs. For most women who choose hair removal therapy, we suggest laser/photoepilation.
Topics: Androgen Antagonists; Androgens; Contraceptives, Oral, Combined; Evidence-Based Medicine; Female; Hair Removal; Hirsutism; Humans; Hypoglycemic Agents; Premenopause; Severity of Illness Index
PubMed: 29522147
DOI: 10.1210/jc.2018-00241 -
Journal of Neuroendocrinology Jun 2023Polycystic ovary syndrome (PCOS) is a female endocrine disorder that is associated with prenatal exposure to excess androgens. In prenatally androgenized (PNA) mice that...
Polycystic ovary syndrome (PCOS) is a female endocrine disorder that is associated with prenatal exposure to excess androgens. In prenatally androgenized (PNA) mice that model PCOS, GABAergic neural transmission to and innervation of GnRH neurons is increased. Evidence suggests that elevated GABAergic innervation originates in the arcuate nucleus (ARC). We hypothesized that GABA-GnRH circuit abnormalities are a direct consequence of PNA, resulting from DHT binding to androgen receptor (AR) in the prenatal brain. However, whether prenatal ARC neurons express AR at the time of PNA treatment is presently unknown. We used RNAScope in situ hybridization to localize AR mRNA (Ar)-expressing cells in healthy gestational day (GD) 17.5 female mouse brains and to assess coexpression levels in specific neuronal phenotypes. Our study revealed that less than 10% of ARC GABA cells expressed Ar. In contrast, we found that ARC kisspeptin neurons, critical regulators of GnRH neurons, were highly colocalized with Ar. Approximately 75% of ARC Kiss1-expressing cells also expressed Ar at GD17.5, suggesting that ARC kisspeptin neurons are potential targets of PNA. Investigating other neuronal populations in the ARC we found that ~50% of pro-opiomelanocortin (Pomc) cells, 22% of tyrosine hydroxylase (Th) cells, 8% of agouti-related protein (Agrp) cells and 8% of somatostatin (Sst) cells express Ar. Lastly, RNAscope in coronal sections showed Ar expression in the medial preoptic area (mPOA), and the ventral part of the lateral septum (vLS). These Ar-expressing regions were highly GABAergic, and 22% of GABA cells in the mPOA and 25% of GABA cells in the vLS also expressed Ar. Our findings identify specific neuronal phenotypes in the ARC, mPOA, and vLS that are androgen sensitive in late gestation. PNA-induced functional changes in these neurons may be related to the development of impaired central mechanisms associated with PCOS-like features.
Topics: Humans; Mice; Female; Pregnancy; Animals; Androgens; Receptors, Androgen; Kisspeptins; Arcuate Nucleus of Hypothalamus; Gonadotropin-Releasing Hormone; GABAergic Neurons; Polycystic Ovary Syndrome; Brain; Virilism
PubMed: 37280378
DOI: 10.1111/jne.13302 -
The Journal of the Association of... Aug 2019Hirsutism is excess terminal hair that commonly appears in a male pattern in women. It is associated with hyperandrogenemia. Ferriman-Gallwey scoring system is the most...
Hirsutism is excess terminal hair that commonly appears in a male pattern in women. It is associated with hyperandrogenemia. Ferriman-Gallwey scoring system is the most popular scoring system for evaluation, treatment and monitoring the response to therapy. Causes include PCOS, Cushing syndrome, glucocorticoid resistance, drugs, more serious conditions like androgen secreting tumour of ovaries or the adrenal gland, hyperthecosis ovarii and luteoma of pregnancy. We present a case of severe PCOS (Hyperthecosis ovarii) which mimics androgen secreting tumour from ovary in a 30- yr- old lady. This case emphasizes on the spectrum of manifestations that PCOS can come with and the importance of trans vaginal ultrasonography in diagnosing ovarian conditions and its superiority over conventional trans abdominal USG or CT scan.
Topics: Adrenal Glands; Adult; Androgens; Female; Hirsutism; Humans; Polycystic Ovary Syndrome; Pregnancy
PubMed: 31562730
DOI: No ID Found -
Journal of Neuroendocrinology Mar 2024Excess levels of circulating androgens during prenatal or peripubertal development are an important cause of polycystic ovary syndrome (PCOS), with the brain being a key...
Excess levels of circulating androgens during prenatal or peripubertal development are an important cause of polycystic ovary syndrome (PCOS), with the brain being a key target. Approximately half of the women diagnosed with PCOS also experience metabolic syndrome; common features including obesity, insulin resistance and hyperinsulinemia. Although a large amount of clinical and preclinical evidence has confirmed this relationship between androgens and the reproductive and metabolic features of PCOS, the mechanisms by which androgens cause this dysregulation are unknown. Neuron-specific androgen receptor knockout alleviates some PCOS-like features in a peripubertal dihydrotestosterone (DHT) mouse model, but the specific neuronal populations mediating these effects are undefined. A candidate population is the agouti-related peptide (AgRP)-expressing neurons, which are important for both reproductive and metabolic function. We used a well-characterised peripubertal androgenized mouse model and Cre-loxP transgenics to investigate whether deleting androgen receptors specifically from AgRP neurons can alleviate the induced reproductive and metabolic dysregulation. Androgen receptors were co-expressed in 66% of AgRP neurons in control mice, but only in <2% of AgRP neurons in knockout mice. The number of AgRP neurons was not altered by the treatments. Only 20% of androgen receptor knockout mice showed rescue of DHT-induced androgen-induced anovulation and acyclicity. Furthermore, androgen receptor knockout did not rescue metabolic dysfunction (body weight, adiposity or glucose and insulin tolerance). While we cannot rule out developmental compensation in our model, these results suggest peripubertal androgen excess does not markedly influence Agrp expression and does not dysregulate reproductive and metabolic function through direct actions of androgens onto AgRP neurons.
Topics: Animals; Female; Humans; Mice; Pregnancy; Agouti-Related Protein; Androgens; Dihydrotestosterone; Mice, Knockout; Neurons; Obesity; Peptides; Polycystic Ovary Syndrome; Receptors, Androgen; Virilism
PubMed: 38344844
DOI: 10.1111/jne.13370 -
Clinical Obstetrics and Gynecology Sep 1987
Review
Topics: Androgens; Child; Female; Humans; Virilism
PubMed: 3308261
DOI: 10.1097/00003081-198709000-00028 -
Obstetrics and Gynecology Clinics of... Dec 1987Hyperandrogenism is not infrequently encountered in infertile women. However, elevated levels of androgen in blood may or may not be associated with hirsutism.... (Review)
Review
Hyperandrogenism is not infrequently encountered in infertile women. However, elevated levels of androgen in blood may or may not be associated with hirsutism. Therefore, the presence of hirsutism alone may not be adequate to rule out hyperandrogenism as a factor in infertility. Although most patients with hyperandrogenism will have menstrual irregularity, some patients may give a normal menstrual history. Specific markers in blood may be used to direct therapy. Not all suppressive therapy is appropriate for use in the infertile patient.
Topics: Acne Vulgaris; Adrenal Gland Diseases; Androgens; Female; Hirsutism; Humans; Infertility, Female; Ovarian Neoplasms
PubMed: 2965328
DOI: No ID Found -
Human Reproduction Update 2012Hirsutism, defined by the presence of excessive terminal hair in androgen-sensitive areas of the female body, is one of the most common disorders in women during... (Review)
Review
BACKGROUND
Hirsutism, defined by the presence of excessive terminal hair in androgen-sensitive areas of the female body, is one of the most common disorders in women during reproductive age.
METHODS
We conducted a systematic review and critical assessment of the available evidence pertaining to the epidemiology, pathophysiology, diagnosis and management of hirsutism.
RESULTS
The prevalence of hirsutism is ~10% in most populations, with the important exception of Far-East Asian women who present hirsutism less frequently. Although usually caused by relatively benign functional conditions, with the polycystic ovary syndrome leading the list of the most frequent etiologies, hirsutism may be the presenting symptom of a life-threatening tumor requiring immediate intervention.
CONCLUSIONS
Following evidence-based diagnostic and treatment strategies that address not only the amelioration of hirsutism but also the treatment of the underlying etiology is essential for the proper management of affected women, especially considering that hirsutism is, in most cases, a chronic disorder needing long-term follow-up. Accordingly, we provide evidence-based guidelines for the etiological diagnosis and for the management of this frequent medical complaint.
Topics: Androgens; Female; Hair Follicle; Hirsutism; Humans; Polycystic Ovary Syndrome; Societies, Medical
PubMed: 22064667
DOI: 10.1093/humupd/dmr042 -
Postgraduate Medical Journal Apr 1975Hirsutism is usually caused either by an increase in androgen production by the ovary or adrenal or it may be due to increased sensitivity of the hair follicle to normal...
Hirsutism is usually caused either by an increase in androgen production by the ovary or adrenal or it may be due to increased sensitivity of the hair follicle to normal amounts of circulating androgens. The diagnostic possibilities can be resolved on clinical grounds by laparoscopy and by hormone measurements. The commonest causes of this symptom are ‘idiopathic hirsutism’ and the polycystic ovary syndrome. Treatment is of the underlying condition or, when that is not possible, by local removal of hair or by the administration of oral contraceptives or glucocorticoids.
Topics: Adrenal Glands; Androgens; Female; Hirsutism; Humans; Ovary
PubMed: 1197153
DOI: 10.1136/pgmj.51.594.236 -
Nederlands Tijdschrift Voor Geneeskunde Oct 2007--Hirsutism, defined as excessive growth of terminal hair in a male-type pattern, is prevalent in women and is most often a feature of androgen excess, associated with... (Review)
Review
--Hirsutism, defined as excessive growth of terminal hair in a male-type pattern, is prevalent in women and is most often a feature of androgen excess, associated with polycystic ovary syndrome (PCOS). Idiopathic hirsutism is the second most common cause. In a small minority of cases, hirsutism is the result of a serious adrenal or ovarian disorder. --The primary aim of diagnostic procedures is to establish or exclude underlying pathology. In the case ofhirsutism, i.e. a modified Ferriman and Gallwey score > 6, analysis of circulating androgen levels and other relevant endocrine parameters is indicated. --Adrenal or ovarian pathologies require primary therapy. --Hirsutism in women with PCOS, or without measurable changes in plasma androgen levels, can be treated with drugs either inhibiting ovarian or adrenal androgen production, blocking androgen receptors or inhibiting 5alpha-reductase in the hair follicle. --Spironolactone or cyproterone acetate combined with an oral contraceptive is the safest and most effective therapeutic approach. --Weight loss is an important part of the treatment of obese, hirsute women with PCOS.
Topics: Androgen Antagonists; Androgens; Contraceptives, Oral; Cyproterone Acetate; Female; Hirsutism; Humans; Obesity; Polycystic Ovary Syndrome; Spironolactone; Treatment Outcome; Weight Loss
PubMed: 18064932
DOI: No ID Found