Signs Of Virilization Research Articles

6450 Leydig Cell Tumor: A Rare Cause of Post-menopausal Hyperandrogenism

Abstract Disclosure: R. Nakdali: None. S. Athimulam: None. Introduction: Ovarian steroid cell tumors are a rare subtype of sex-cord stromal tumors. Leydig cell tumors is a subtype of sex-stromal tumors that is found in < 1% of all ovarian tumors. They are typically benign, unilateral, and secrete androgens which causes virilization. Below we present the case of a postmenopausal woman who presented with signs of virilization and was diagnosed with an ovarian Leydig cell tumor. Case: A 62-year-old postmenopausal woman, with a history of partial hysterectomy with right oophorectomy presented to Endocrinology clinic for evaluation of thyroid nodules. However, clinically she had signs of virilization noted by the provider on examination. She reported progressive androgenic alopecia, cystic acne, oily skin, hoarseness of voice and hirsutism, with increase hair growth over upper lip, chin and jaw line requiring daily shaving. She denied taking any supplements. She reported embarrassment of these physical changes and prior providers had attributed this to aging, therefore not prompting further testing. This caused significant emotional distress to the patient, leading to isolation. Biochemical testing confirmed elevated bioavailable testosterone (193.1 ng/dL) and total testosterone levels (579 ng/dL), with no evidence of hypercortisolemia. Magnetic resonance imaging (MRI) revealed a heterogenous soft tissue mass (2.9 x 2.1 x 2.1 cm) in the left ovary. She underwent a left salpingo-oophorectomy and pathology confirmed a well-differentiated 2.4 cm Leydig cell tumor. Post-operatively, she reported improvement in facial and body hirsutism, reduced shaving frequency decrease in androgenic hair loss, with noticeable hair growth in her frontal scalp, and her skin became less oily with reduced acne. Post-operative labs confirmed cure of hyperandrogenism (Bioavailable testosterone < 2.6 ng/dL; Total testosterone: < 10ng/dL; and Free androgen index < 0.5%). She has been referred to genetics for evaluation. Discussion: Post-menopausal hyperandrogenism poses a diagnostic challenge as it can be mistaken for hormonal imbalances seen with aging. Often times, patients do not report symptoms due to embarrassment unless specifically addressed by provider. Initial testing consists of total and free testosterone and dehydroepiandrosterone sulfate (DHEAS) levels to assess the source of hyperandrogenism. Ruling out hypercortisolemia and assessment for ingestion of testosterone or DHEA supplements is vital. Pelvic ultrasound or cross-sectional imaging (CT or MRI) of abdomen and pelvis can identify structural abnormalities in the ovaries or adrenal glands. Prompt evaluation and management can alleviate significant distress to the patient. Leydig cell tumors represent a rare but important consideration in the differential diagnosis of postmenopausal hyperandrogenism, such as in our patient. Presentation: 6/2/2024

6450 Leydig Cell Tumor: A Rare Cause of Post-menopausal Hyperandrogenism

Abstract Disclosure: R. Nakdali: None. S. Athimulam: None. Introduction: Ovarian steroid cell tumors are a rare subtype of sex-cord stromal tumors. Leydig cell tumors is a subtype of sex-stromal tumors that is found in < 1% of all ovarian tumors. They are typically benign, unilateral, and secrete androgens which causes virilization. Below we present the case of a postmenopausal woman who presented with signs of virilization and was diagnosed with an ovarian Leydig cell tumor. Case: A 62-year-old postmenopausal woman, with a history of partial hysterectomy with right oophorectomy presented to Endocrinology clinic for evaluation of thyroid nodules. However, clinically she had signs of virilization noted by the provider on examination. She reported progressive androgenic alopecia, cystic acne, oily skin, hoarseness of voice and hirsutism, with increase hair growth over upper lip, chin and jaw line requiring daily shaving. She denied taking any supplements. She reported embarrassment of these physical changes and prior providers had attributed this to aging, therefore not prompting further testing. This caused significant emotional distress to the patient, leading to isolation. Biochemical testing confirmed elevated bioavailable testosterone (193.1 ng/dL) and total testosterone levels (579 ng/dL), with no evidence of hypercortisolemia. Magnetic resonance imaging (MRI) revealed a heterogenous soft tissue mass (2.9 x 2.1 x 2.1 cm) in the left ovary. She underwent a left salpingo-oophorectomy and pathology confirmed a well-differentiated 2.4 cm Leydig cell tumor. Post-operatively, she reported improvement in facial and body hirsutism, reduced shaving frequency decrease in androgenic hair loss, with noticeable hair growth in her frontal scalp, and her skin became less oily with reduced acne. Post-operative labs confirmed cure of hyperandrogenism (Bioavailable testosterone < 2.6 ng/dL; Total testosterone: < 10ng/dL; and Free androgen index < 0.5%). She has been referred to genetics for evaluation. Discussion: Post-menopausal hyperandrogenism poses a diagnostic challenge as it can be mistaken for hormonal imbalances seen with aging. Often times, patients do not report symptoms due to embarrassment unless specifically addressed by provider. Initial testing consists of total and free testosterone and dehydroepiandrosterone sulfate (DHEAS) levels to assess the source of hyperandrogenism. Ruling out hypercortisolemia and assessment for ingestion of testosterone or DHEA supplements is vital. Pelvic ultrasound or cross-sectional imaging (CT or MRI) of abdomen and pelvis can identify structural abnormalities in the ovaries or adrenal glands. Prompt evaluation and management can alleviate significant distress to the patient. Leydig cell tumors represent a rare but important consideration in the differential diagnosis of postmenopausal hyperandrogenism, such as in our patient. Presentation: 6/2/2024

Benign pure androgen-secreting adrenal tumor misdiagnosed as adrenocortical carcinoma on 18F-FDG PET-CT: a rare case report.

Adult pure androgen-secreting adrenal tumors (PASATs) are rarely reported and the malignancy of such tumor are difficult to confirm before surgery. Here we report a PASAT demonstrating extremely 18F-FDG uptake turned out to benign tumor by postoperative pathology examination. A 19-year-old adolescent found a tumor measuring 7.2 cm located in the right adrenal region on enhanced CT during the routine physical examination. Signs of virilization and elevated testosterone and dehydroepiandrosterone (DHEA) were verified during preoperative examination. 18F-FDG PET/CT revealed the tumor had an extremely high 18F-FDG uptake with a SUVmax reaching 42.7, which turned out to be oncocytic adrenocortical adenoma by pathological examination. 18F-FDG PET/CT may be limited in assessing the malignancy of PASAT.

Atypical Congenital Adrenal Hyperplasia (CAH) Presentation of A Patient, Resulting in A Similar Diagnosis for His Sibling

Introduction: Congenital adrenal hyperplasia (CAH) is the commonest cause of primary adrenal insufficiency and is known to be transmitted in an autosomal recessive manner. We report two cases of male siblings with CAH. Cases: The elder sibling was diagnosed late at 4 years old with signs of virilization, complicated with central precocious puberty (CPP). The younger sibling was diagnosed after baseline screening and stimulation test. Treatment with both hydrocortisone and fludrocortisone were commenced for both siblings once the diagnosis was made. The elder sibling however, required treatment with gonadotropin analogues to treat the CPP. Conclusion: These cases illustrate the importance of an early diagnosis of CAH and the benefit of screening siblings to prevent late presentations of CAH in high-risk families.

Selective peripheral tissue response to high testosterone levels in an infertile woman without virilization signs.

Total testosterone, which is peripherally converted to its biologically active form dihydrotestosterone (DHT), is the first-line hormone investigation in hyperandrogenic states and infertility in premenopausal women. Polycystic ovary syndrome (PCOS), the most common cause of hyperandrogenism and infertility in young women, is often associated with mild elevations of total testosterone. Whereas very high levels of total testosterone (>2-3 SD of normal reference), are most often associated with hyperandrogenic signs, menstrual irregularity, rapid onset of virilization, and demand a prompt investigation. Herein, we report a case of a 32-year-old woman who was referred to the endocrinology outpatient clinic due to secondary amenorrhea and extremely high testosterone levels without any virilization signs. We initially suspected pitfalls in the testosterone laboratory test. Total serum testosterone decreased after a diethyl-ether extraction procedure was done prior to the immunoassay, but testosterone levels were still elevated. An ovarian steroid-cell tumor (SCT) was then revealed, which was thereby resected. Twenty-four hours post surgery, the total testosterone level returned to normal, and a month later menstruation resumed. This case emphasizes that any discrepancy between laboratory tests and the clinical scenario deserves a rigorous evaluation to minimize misinterpretation and errors in diagnosis and therapeutic approach. Additionally, we describe a possible mechanism of disease: a selective peripheral target-tissue response to high testosterone levels that did not cause virilization but did suppress ovulation and menstruation. Total testosterone is the most clinically relevant hormone in investigating hyperandrogenic states and infertility in premenopausal women. Very high total testosterone levels in women (>2-3 SD of normal reference) are most often associated with hyperandrogenic signs, menstrual irregularities, and a rapid onset of virilization. In women with very elevated testosterone levels and the absence of clinical manifestations, laboratory interference should be suspected, and diethyl ether extraction is a useful technique when other methods fail to detect it. Ovarian steroid cell tumors (SCT) encompass a rare subgroup of sex cord-stromal tumors and usually secrete androgen hormones. SCTs are clinically malignant in 25-43% of cases. A selective response of peripheral target tissues to testosterone levels, with clinical manifestations in some tissues and no expression in others, may reflect differences in the conformation of tumor-produced testosterone molecules.

R Representasi Maskulinitas Pada Tokoh Dola dalam Anime Laputa: Castle in the Sky (1986)

This research aims to describe the representation of masculinity in the Dola character in the anime "Laputa: Castle in the Sky (1986)." This study is a linguistic research employing a qualitative descriptive method. The research theory used includes Roland Barthes' semiotics and the concept of masculinity by David Deborah and Robert Brannon. Data collection was conducted through observation and note-taking, and data validity was ensured through data triangulation. The findings of this study indicate that the character Dola reflects masculinity. Various signs of masculinity are evident through Dola's character design, which includes dominance, protectiveness, the ability to separate feelings from ideas, aggressiveness, ambition, physical strength, rationality, leadership qualities, daringness, competence and competitiveness. Despite embodying masculinity, Dola maintains feminine aspects in her appearance, as reflected in her wearing of a skirt, two braided pink hair, the use of earrings, and her tender-heartedness displayed through her affection for Sheeta and Pazu.
 

Peculiarities of the formation of psychological signs of masculinity and femininity in athletes (on the example of sports acrobatics)

The article presents a study of determining the degree of manifestation of inversion of sexual dimorphism using the study of psychological types (behavioral indicators of masculinity/femininity) according to the method of Sandra Bem (S. Bem) in acrobats of various roles and genders and non- athletes. Athletes specializing in pair and group sports acrobatics and persons of the same age who do not play sports took part in the research. Acrobatic girls were divided into 2 groups: 1) roles at the top (n=32), puberty age (13.96±1.02), 2) roles in the middle and at the bottom (n=42), youth age (19.36±2.86). Young acrobats: 1) roles above (n=32), average age 14.88±2.32; 2) roles in the middle and below (n=48), average age 20.38±2.89. Sports qualification of athletes: beginners, sub-elite and elite athletes. All subjects were offered the method of determining a person's gender identification (psychological sex) according to S. Bem, which is based on determining the degree of masculinity, femininity and androgyny of an individual. It was found that 10% of female respondents, 12-25 years old (the roles above), specializing in pair-group acrobatics, belong to the masculine type of gender identity, and 90% to the andromorphic type. In those who do not do sports: masculinity – 3%, femininity – 10%, androgyny – in 87% of the subjects. 45% of male acrobats showed signs of masculinity, 55% – androgyny. In people who do not do sports: masculinity – 30%, androgyny – 70%. Acrobats aged 16-23 (the roles in the middle and below: masculinity – 20%, androgyny – 80%. Feminine personality type was not found in any of the athletes. In female acrobats of both roles, the test data obtained using the psychological gender method indicate a shift from the feminine type of gender identification to the masculine and androgynous types.

SAT322 Case report: Impact Of Combined Treatment To Prolong Overall Survival In Advanced Adrenocortical Pediatric Cancer Associated With Li-Fraumeni Syndrome

Abstract Disclosure: I.P. de Magalhães: None. N.C. de Sousa: None. H.L. Charchar: None. A.W. Kuhn: None. M.Y. Nishi: None. F.L. Ledesma: None. R.I. Lopes: None. F.T. Dénes: None. P. Herman: None. M.R. Menezes: None. A. Latronico: None. B.B. Mendonca: None. M.Q. Almeida: None. M.B. Fragoso: None. Background: Adrenocortical cancer (ACC) in pediatric patients (<15 years of age) are even rarer compared to the general population it is a classic presentation of Li-Fraumeni syndrome (LFS). ACC incidence in this subgroup is limited to 0.3 cases per million per year with a high incidence in Sothwest of Brazil. Furthermore, the overall five-year survival of pediatric ACC cases is better than in adult with a rate of approximately 50%, considering a complete resection required for cure. However, prognosis of childhood ACC is highly variable and difficult to predict in clinical practice. Clinical Case: We present the case of a 3-year-old girl who was diagnosed with metastatic adrenocortical carcinoma with classical signs of virilization and Cushing's syndrome. The patient underwent transperitoneal right adrenalectomy in May 2011. During surgery, there was rupture of the tumor capsule and the inferior vena cava. She was referred to our service after 8 months due to the presence of locoregional recurrence and maintained hormonal secretion. Histological data confirmed ACC and the tumor was classified as Weiss score 5, Wieneke: 4, Ki67 40%. Genetic analysis identified germline confirmed p-R337H on TP53, XAF1-E134. Imaging exams documented suspected liver metastasis and peritoneal implants. She underwent palliative treatment with mitotane (M) and systemic chemotherapy EDP+M with partial response. A new surgical approach to the residual lesions was proposed. The patient underwent right partial hepatectomy and peritoneal implant removed in September 2012. After metastasectomy, the patient presented persistence of the disease. The patient was exposed to a new cycle of EDP+M, but the disease progressed. She underwent radiofrequency ablation of abdominal lesions in July 2013 without success. A new surgical approach was indicated in July 2013. The histological analysis confirmed metastases of ACC. She underwent a new chemotherapy regimen with gemcitabine, docetaxel and fluorouracil + M. She presented a complete response confirmed by radiological exams and hormonal data. Mitotane was discontinued in 2016 due to bladder bleeding. She presented an excellent response with combined treatment, without recurrence up to now with 10 years of recurrence free survival and 12 years of overall survival. She developed normal puberty and reached the target height and maintains primary adrenal insufficiency with glico and mineralocorticoid replacement. Although the tumor spillage, histological and molecular data predicted a dismal outcome this patient surprisingly is presenting an excellent evolution under continuous surveillance. Clinical Lesson: This report described a challenging pediatric advanced ACC which the combined treatment strategy provided successful outcome Presentation: Saturday, June 17, 2023

Estimation of Reference Values for External Genitalia Parameters in North Indian Preterm and Term Female Newborns.

To establish gestation-wise normative data of external genitalia measurements in North Indian term and preterm female newborns. In this cross-sectional descriptive study, institutionally-born female neonates between 28-42 wk gestation were consecutively enrolled between 24-72 h of life. Newborns with major congenital malformations, chromosomal anomalies, multifetal gestation and birth injuries were excluded. Data on various genital measurements were collected [Clitoral length (CL), clitoral width (CW), ano-clitoral distance (AGDAC), ano-fourchette distance (AGDAF) and anogenital ratio (AGR)]. One hundred ninety-eight of 508 neonates (39%) were preterm and 310 (61%) were term. Mean (± SD) CL and CW were 4.6 ± 1.8 mm and 3.9 ± 1.6 mm, respectively. Mean (± SD) values for AGDAF, AGDAC and AGR were 9.3 ± 1.8 mm, 30.2 ± 3.9 mm, and 0.31 ± 0.05, respectively. According to these results, term female newborns with CL more than 7 mm and/or CW more than 6 mm (95th centile) warrant evaluation for clitoromegaly. An anogenital ratio greater than 0.45 should be considered as a sign of virilization in a female neonate. Gestation-wise percentile charts for CL, CW, AGDAF, AGDAC and AGR were generated. The percentile values defined in the study can serve as local normative data for accurate interpretation of genital measurements in North Indian female newborns and enable health care professionals for early identification of genital virilization.

Extremely high testosterone level in a woman without virilization signs. Is it only laboratory pitfalls?

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Virginity as a sign of Masculinity: The case of the Borana Oromo, Ethiopia

This article discusses the socially constructed masculinity of unmarried girls among the Borana in southern Ethiopia. The Borana practice the longstanding Gadaa political system, which was inscribed by UNESCO as an intangible cultural heritage of humanity in 2016. Data for this article was gathered through qualitative approaches, including interviews, focus group discussions (FGDs), and direct observation of the 2012 Gadaa general assembly. Such an assembly is held once every eight years to enact new Gadaa rules and amend the existing ones. The findings indicated that the Gadaa system provides rules regulating Borana marriage lives, including premarital virginity for girls. The Borana refers to an unmarried girl as “dubra gammee,” which means “a virgin girl,” who has a masculine identity and is “a male person.” Unmarried girls have certain physical markers, particularly a hairstyle that they share with boys. Any sexual act with unmarried girls is therefore homosexual and punishable. Gadaa and all related institutions care for the premarital virginity of girls. The Gadaa rules impose punishment unconditionally on men who have sex with unmarried girls. On the other hand, the Borana tolerate extramarital sexual relations, which has partly contributed to maintaining the premarital sexual innocence of Borana girls.

Congenital adrenal hyperplasia in adolescence - a gynecological perspective.

Analysis of congenital adrenal hyperplasia (CAH) cases, gynaecological implications, referral reasons to gynaecologist and treatment. Retrospective, longitudinal, single-centre study with female CAH paediatric patients ≥ 10 years-old, followed between 1998-2018 in gynaecology and endocrinology departments at a public university tertiary hospital. 47 patients, 34.0% (n = 16) with classic, 66.0% (n = 31) with non-classic forms (NCAH), CYP21 deficit and 46,XX karyotype. We found a normal median menarche age (11.5 IQR 2 [6-15] years-old), but significantly earlier in NCAH (p = 0.003). Precocious puberty occurred in 48.9%, n = 23. Primary amenorrhea occurred in salt-wasting form (21.4%, n = 3). Oligomenorrhea and hirsutism were significantly more prevalent in NCAH (p = 0.018, p = 0.014 respectively) and acanthosis nigricans and virilization signs in classic forms (p = 0.05, p = 0.000 respectively). Sixteen patients (34.0%) were referred to gynaecology, mostly due to menstrual irregularities (50.0%, n = 8). Medical treatment with isolated or combined corticoids, oestrogen and progestogen were chosen in all but one case. Gonadotropin-releasing hormone analogues were used in 19.0% (n = 9). Surgery was performed in 34.0% (n = 16) patients, median age 2.0 IQR 2.5 (0.6-90) years-old. This paper highlights the importance of a multidisciplinary approach. Early treatment contributes to a phenotypical feminine differentiation and normalization of the hypothalamus-pituitary-ovarian axis, which is essential given the gynaecologic and obstetric consequences of untreated cases.

Laparoscopic assisted removal of large ovarian mass causing precocious puberty in an infant—a case report

BackgroundSex cord and stromal tumors are a heterogeneous group of tumors that arise from gonadal sex cord cells, gonadal stromal cells, or both. They are divided into pure stromal tumors, pure sex cord tumors, and mixed tumors. Some of these tumors are hormonally active, producing androgens and estrogens, and may therefore exhibit virilization or excess estrogen. Sertoli-Leydig cell tumors are rare tumors belonging to mixed tumors representing less than 0.5% of ovarian tumors. Few cases have been reported in the medical literature.Case presentationWe report the case of a 1-year-old girl who was admitted for breast lumps and pubic hair with intermittent painless vaginal bleeding in the past 2 weeks. The abdominal examination objectified the presence of an intraperitoneal mass to which an abdominal ultrasound and computerized tomography (CT) scan had confirmed the presence of a right ovarian mass. A laparoscopy was performed which showed an encapsulated mass arriving from the right ovary replacing the normal ovarian tissue. Laparoscopic-assisted removal of the entire mass was done. The postoperative course was uneventful. The histopathological study demonstrated an intermediately differentiated Sertoli cell tumor with an intact capsule, no lymphovascular invasion, or heterologous elements (TNM stage 1).ConclusionsAlthough Sertoli-Leydig tumors are commonly seen after the second decade of life in young women, their discovery before puberty is not uncommon. Mass syndrome and signs of virilization are two frequently reported signs that should prompt the clinician to mention this entity among the differential diagnoses. The first-line radiological examination is pelvic ultrasound. The treatment is mainly based on surgery. The prognosis is excellent especially if the tumor is well differentiated.

Promene nivoa polnih hormona sa starenjem i njihov uticaj na glas

The aim of this study is to review modern literature to provide an insight into contemporary research into the etiology, pathophysiology and symptomatology of changes in hormone levels associated with aging and their pattern-consequential relationship with voice quality, with special reference to the analysis of different types of mutations (mutatio precox and mutatio perversa), menstrual and climacteric dysphonia. The criteria for inclusion of works obtained from the search were: works published during the last 15 years, published in English. Compared to the male voice, the female voice does not go through drastic changes during puberty. In males, the increased levels of testosterone and dihydrotestosterone during puberty are responsible for the enlargement of the laryngeal cartilage, which causes the basal tone to decrease by a third of an octave. If the child enters puberty prematurely, this hormone imbalance will lead to the appearance of voice pathology -mutation precox. Mutatio perversa occurs suddenly in females and is characterized by a deep male voice and other signs of virilization. The appearance of submucosal bleeding in the period before menstruation and the increased secretion of estrogen that leads to the appearance of edema will cause premenstrual dysphonia. Voice quality in this period is characterized by vocal fatigue, reduced voice range with loss of high tones, loss of vocal power and hoarseness of voice. Menopausal vocal syndrome is characterized by reduced flexibility and a reduced range of vibrational amplitude. It can be concluded that the vocal cords are a target organ, sensitive to fluctuations in the level of sex hormones due to the physiological aging process and the resulting endocrine disorders during life.

Rare virilizing tumor: ovarian steroid cell tumor, not otherwise specified: a case report

BackgroundOvarian steroid cell tumors, not otherwise specified is a rare sex cord-stromal tumor. Almost 60% of all steroid cell tumors are categorized as not otherwise specified and represent less than 0.1% of all ovarian neoplasm. Some of them are endocrinologically active, producing virilization signs in young women. The recommended treatment is primarily surgical.Case presentationWe present the case of a 20-year-old Mexican woman with secondary amenorrhea and virilization signs. She was treated with combined oral contraceptives from 13 years old, due to a misdiagnosis of polycystic ovarian syndrome. However, 4 months after stopping medication, amenorrhea and virilization signs worsened. Biochemically, she had high serum total testosterone and free testosterone levels, and a pelvic and transvaginal ultrasound followed by a pelvic tomography scan demonstrated a right adnexal tumor. She underwent right salpingo-oophorectomy and the histopathological and immunochemistry exams confirmed the diagnosis. The patient was followed for a year after surgery and until then, her menses were regular and she had no recurrence of virilization signs. ConclusionThe purpose of this case report is to alert physicians to rule out ovarian steroid cell tumor, not otherwise specified diagnosis in young women with increased testosterone after discarding common causes such as polycystic ovarian syndrome. A multidisciplinary team including a gynecologist, endocrinologist, radiologist, and pathologist should be involved for correct diagnosis at the proper time.

ODP375 Absence of Virilization With Completely Normal Phenotype in a Newborn Female With Salt-Wasting Classical CAH

Abstract Background Congenital adrenal hyperplasia (CAH) is an autosomal recessive disorder that leads to the disruption of adrenal steroidogenesis. 21-hydroxylase deficiency is the most common form and is caused by the mutations in the 21-hydroxylase (CYP21A2) gene. Deficiency in the 21-hydroxylase enzyme can affect aldosterone and cortisol production and shift the pathway to increased androgen production. It is classified as classical and nonclassical depending on the severity of enzyme deficiency. The classical form can present as a life-threatening salt-wasting or a milder form of simple virilizing CAH. Female patients with classical 21-hydroxylase deficiency present with ambiguous genitalia secondary to increased androgen production. We present a case of salt-wasting classical CAH in a female patient with no signs of virilization that was diagnosed based on newborn screening. Case presentation A four-day-old full-term female was referred to our hospital for elevated 17 hydroxyprogesterone (17-OHP) on a newborn screen. She was born via normal vaginal delivery with an uncomplicated prenatal and postnatal course. Her newborn screen was drawn at day 1 of life resulted in 17-OHP of 128.20 nmol/L (normal cutoff of 85 nmol/L). Otherwise, she was active, feeding well with no other concerns. On admission, her vital signs were within normal, with an unremarkable physical examination. Specifically, the genitourinary exam demonstrated normal female external genitalia with no evidence of virilization, no clitoromegaly with a clitoris length of 12 mm and width 1-2 mm, no hyperpigmentation, and with normal-appearing vaginal orifice and urethral meatus. Confirmatory 17-OHP by mass spectrometry was sent along with the metabolic panel, and her initial electrolytes were within normal. Since there was a low suspicion for salt-wasting CAH given her normal labs and unremarkable physical exam, medication was not started, and the patient was admitted for monitoring. The potassium level trended up abnormally to 6.3 mmol/L, which prompted the initiation of empiric treatment with a physiologic dose of Hydrocortisone and Florinef on day of life 7, pending confirmatory 17-OHP level. Confirmatory17-OHP was 1242 ng/dL (normal <78) and following stimulation with Cosyntropin a markedly elevated 17-OHP 27,929.31 ng/dL (normal expected 7-106) and low cortisol level 2 mcg/dL, for which definitive treatment with hydrocortisone and Florinef were initiated. Subsequent genetic testing confirmed the diagnosis of 21-hydroxylase deficiency with mutations in the CYP21A2 gene for I172N, R356W, and c. *13G>A. Further genetic workup to explain the complete lack of virilization, including assessment of an androgen insensitivity panel and whole-exome sequencing, was unremarkable. Conclusion This case illustrates the critical importance of heel-stick newborn screening for CAH to prevent a life-threatening salt-wasting crisis as female patients with classical CAH can present with a phenotypic complete lack of virilization that could be misleading and fatal if not recognized. Presentation: No date and time listed

Pediatric adrenal incidentaloma as a rare and difficult to diagnose finding - review

Adrenal incidentaloma (AI) is an asymptomatic mass localized in the adrenal gland, usually detected during diagnostic imaging performed due to other health issues than the suspected adrenal disease. It occurs in 2% of adults and 0,2% of children. Pediatric AI may cause many difficulties in the diagnostic processes. Neuroblastoma is one of the most common malignant tumors in children. Usually, it is localized in the medulla of the adrenal gland. Many patients do not have any symptoms, but some may present with fever, weariness, weight loss, pain, enlarging mass, etc. The majority of cases are high- risk and the survival rate is less than 50%. The diagnostic protocol consists of laboratory tests and radiological imagining. Treatment depends on the risk group that the patient is assigned to. Pheochromocytoma is a rare catecholamine-secreting tumor in children originating in the adrenal medulla. Most pediatric patients present with sustained hypertension. 60% of pheochromocytoma cases are sporadic. The surgical removal of the tumor through adrenalectomy is a standard procedure. To the group of pediatric adrenocortical tumors (ACT), benign adrenocortical adenoma (ACA) and malignant adrenocortical carcinoma (ACC) are included. ACA is found in only 20% of cases of ACT. Most of the patients present with signs of virilization, Cushing Syndrome, or both. The 5-years survival rate of ACC in the pediatric population is 46-55%. An adrenal incidentaloma is an unpredictable and possibly dangerous disorder in the pediatric population. Due to the rarity of the finding and their variety, there is not much data and clear recommendations about AI management. Doctors need to know about the prevalence of different types of adrenal tumors in the pediatric population based on age.

Une hyperandrogénie chez la femme ménopausée : origine ovarienne ou origine surrénalienne ?

Postmenopausal hyperandrogenism is an androgen excess originating from either the adrenals and/or the ovaries. Clinically, symptoms can be moderate (increase in terminal hair growth, acnea) or severe with signs of virilization (alopecia, clitoridomegaly). In either setting, physicians need to exclude relatively rare but potentially life-threatening underlying tumorous causes, such as adrenal androgen-secreting tumors. The objectives of this review are to evaluate which hormonal measurements (T, delta 4 androstenedione, 17 OH progesterone, SDHEA, FSH, LH) and/or imaging (pelvic ultrasound, MRI or adrenal CT-scan) could be useful identifying the origin of the androgen excess. Our review illustrates that the rate of progression of hirsutism and/or alopecia, and serum testosterone levels are in favor of tumors. Pelvic MRI and adrenal CT-scan are useful tools for identifying the different causes of androgen excess.

The Masculine and The Feminine in Arabic and the Illusion of the Unnecessary Proliferation of Words

This research investigates masculine and feminine in Arabic, aiming at highlighting the formal and semantic limits set by Arab linguists for each of them. Two further issues constitute the aim of this research: the first: the reality of the formal indicators of feminization, and the extent of their conformity with reality, The second is to explain the cause of the existence of these formal signs and to refute the idea of classical linguists that they came to restrict the proliferation of words. This research aims to clarify these elements associated with the idea of femininity in Arabic, and to indicate whether the masculine was originally feminine or not? And spot some formal masculinization as signs of femininity. The research reached a set of results, most notably that the formal signs of the feminine were not strict to the extent adopted by the classical Arab linguists; some Arabic signs of masculinization are not addressed by the classical linguists. It also concluded that the issue of unnecessary proliferationof words was not a suitable cause for the presence of signs of femininity, as the language is not incapable of generating words, nor burdened by it.

Peli scrotali isolati nel lattante: un segno di allarme o una normale variante?

Isolated scrotal hair development in infancy is a rare disorder that has not been well-characterized. The article describes a case of transient isolated scrotal hairs in a five-month-old boy. Except for scrotal hair development, physical examination was normal. There were no other signs of virilization or secondary sexual development. Growth pattern, laboratory evaluation and bone-age radiographs were unremarkable. Spontaneous disappearance of scrotal hairs observed at the end of the first year of life finds confirmation in the literature that considers the transient isolated scrotal hairs as a rare self-limiting benign condition.