Severe intrahepatic cholestasis of pregnancy due to a Sertoli-Leydig cell tumour in a woman with polycystic ovary syndrome: a case report.

BACKGROUND: Intrahepatic cholestasis of pregnancy (ICP) is a common gestational complication characterized by pruritus and elevated bile acids, usually occurring in the third trimester when the serum estrogen and progesterone levels are highest. Hyperandrogenism during pregnancy is a pathological state that is mostly induced by polycystic ovary syndrome (PCOS) but rarely by concomitant androgen-secreting ovarian tumours. To date, no correlation has been drawn between ICP and hyperandrogenism. CASE PRESENTATION: Here, we present a rare case of early-onset severe ICP in a PCOS patient conceived via in vitro fertilization-embryo transfer, with worsening hirsutism and acne due to high levels of testosterone and dehydroepiandrosterone sulphate, both of which were produced by a fast-growing ovarian Sertoli-Leydig cell tumour. Her serum estradiol was also very high, which was speculated to be converted from the circulating androgens by the placenta. She had preterm premature rupture of membranes and delivered at 30 weeks, followed by a rapid remission of ICP as her serum estradiol dropped. However, the excessive androgens did not retreat until the large ovarian tumour was surgically removed. CONCLUSION: This unusual case highlights the concurrence of original hyperandrogenism and subsequent hyperestrogenism during pregnancy and the resultant confounding manifestations. Obstetricians should be aware of the potential association between androgen excess and ICP via placental aromatization.

Sertoli-Leydig cell ovarian tumour: a rare cause of virilisation and androgenic alopecia.

Sertoli-Leydig cell tumours (SLCTs) represent a rare cause of hyperandrogenic state. SLCTs are sex cord ovarian neoplasms, accounting for <0.2% of all ovarian tumours. Most of the sex cord-stromal tumours have a benign clinical course, with 10%-20% of them at risk of aggressive course. We report a case of a woman in her 30s who presented with androgenic alopecia, virilisation and secondary amenorrhoea. The evaluation revealed an extremely high testosterone level. Imaging for the localisation of source of excess testosterone with contrast-enhanced CT of the abdomen revealed a right ovarian mass. Hence, a diagnosis of testosterone-secreting ovarian tumour was considered. The patient underwent right salphingo-oophorectomy, and histopathology was reported as Sertoli cell tumour. Postoperatively, there was normalisation of serum testosterone levels with decrease in virilisation and resumption of spontaneous menstrual cycles. The patient conceived spontaneously after 2 months of surgery.

From diagnosis to treatment of androgen-secreting ovarian tumors: a practical approach.

About 5% of all ovarian tumors develop some form of hormonal activity. Only 1% of ovarian tumors will secrete androgens causing clinical hyperandrogenism. Most androgen-secreting neoplasms (ASN) derive from sex cord or stroma cells of the ovary and may affect both premenopausal and postmenopausal women. Typically, a patient will present reporting symptoms of rapidly increasing hyperandrogenization such as: hirsutism, acne, frontal/male pattern balding, and in severe cases even virilization. Sertoli-Leydig Cell Tumors are the most frequent ASN and constitute about 0.5% of all ovarian neoplasms. Typically affecting women under 30 years of age, these tumors are usually unilateral and benign. They are also the most common tumor in postmenopausal women suffering with hyperandrogenism. Other tumors originating from the sex-cord stroma are also known to develop in this population, but the incidence of these is much lower. Approaching suspected hyperandrogenemia and its related symptoms in a clinical setting can be a significant diagnostic challenge. When evaluating a patient for hyperandrogenism, it is important to assess the severity of symptoms but most of all it is critical to assess the time of onset and dynamics of symptom progression. Diagnostic tools including laboratory tests and imaging studies should also be engaged. When deriving a differential diagnosis for androgen-secreting ovarian tumors, adrenal gland tumors should be considered as well as typical endocrine pathologies including polycystic ovary syndrome, congenital adrenal hyperplasia, Cushing's disease, and acromegaly. Treatment options for an androgen-secreting ovarian tumors is mainly surgical, but in exceptional cases can involve pharmacotherapy alone.

Moderately differentiated Sertoli-Leydig cell tumor of ovary with associated mucinous carcinoma and carcinoid-A case report and review of literature.

Sertoli-Leydig Cell Tumors (SLCT) are very rare neoplasms of the ovary (0.2%) and they belong to the group of sex cord-stromal tumors. Of these, 20% of the cases show heterologous elements. We report a case of a 22-year-old woman who presented with complaints of lower abdominal pain and secondary amenorrhea for 10 months. Physical examination revealed right lower abdominal tenderness and fullness. Imaging showed a right ovarian mass. She underwent right salpingo-oophorectomy with bilateral pelvic lymphadenectomy and omentectomy. Microscopic examination revealed a neoplasm with varied histomorphological patterns. The predominant pattern was an atypical proliferative mucinous tumor with foci of microinvasion. The other component was that of moderately differentiated Sertoli-Leydig Cell Tumor. Focal areas resembling carcinoid were also noted. Immunohistochemistry was performed and the Sertoli-Leydig Cells were positive for CD56, calretinin, inhibin, vimentin, and ER. The glandular component was positive for CK20, EMA, CEA, and CDX2. Synaptophysin and chromogranin were positive within nests resembling carcinoid. With the given histomorphological features and immunohistochemistry findings, a diagnosis of moderately differentiated Sertoli-Leydig Cell Tumor of the ovary with associated mucinous carcinoma and carcinoid was rendered. The presence of heterologous elements in SLCTs has been reported to be associated with poor prognosis.

Clinicopathological analysis of ovarian sertoli-leydig cell tumor with postmenopausal vaginal bleeding as the first symptom: A case report.

RATIONAL: Ovarian sertoli-leydig cell tumor (OSLCT) is extremely rare. We reported a OSLCT case in whom postmenopausal vaginal bleeding was the first symptom. PATIENT CONCERNS: The patient came to our hospital due to postmenopausal vaginal bleeding. DIAGNOSES: Serum tumor markers and color Doppler ultrasound for her pelvic cavity were negative. The patient was finally diagnosed with left OSLCT by pathology. It was difficult to make a definite diagnosis before operation, the diagnosis of OSLCT required postoperative pathology in the patients. INTERVENTIONS: the patient underwent laparoscopic hysterectomy+bilateral adnexectomy+lysis of pelvic adhesions. OUTCOMES: Postoperative laboratory examinations were normal. The patient was discharged from our hospital on the seventh day after operation and came to our hospital for follow-up check in April 2020. Physical and laboratory examinations were normal. LESSONS: OSLCT can show different endocrine abnormalities, which are related to the various types of tumor tissues. Missed diagnosis and misdiagnosis are likely to occur in the patients who only have elevated serum testosterone. For the menopausal women with elevated serum testosterone, ovarian tumor shoule be highly suspected after excluding adrenal gland-related diseases.

Sertoli-Leydig cell tumour in a patient with non-classic congenital adrenal hyperplasia: an uncommon duo.

Polycystic ovary syndrome is the most common cause of hyperandrogenism in young females. Other causes are congenital adrenal hyperplasia (CAH), androgen-producing tumours and drugs. The severity and tempo of virilisation help in distinguishing the tumoural from non-tumoural causes. We report a rare case of non-classic CAH and androgen-producing ovarian tumour in the same patient, causing hyperandrogenism. A 15-year-old female patient presented with secondary amenorrhea, excessive facial hair growth and clitoromegaly for 6 months. Due to severe virilisation, tumoural aetiology was considered. Investigations showed marked elevation of testosterone and mild elevation of 17 hydroxy progesterone (17OHP). Imaging confirmed right ovarian tumour. Adrenocorticotropic hormone stimulated 17OHP, was elevated confirming the diagnosis of underlying non-classic CAH. Surgical removal of the tumour was followed by improvement in hyperandrogenism, but persistent elevation of 17OHP confirmed the underlying presence of non-classic CAH.

The clinical features and reproductive prognosis of ovarian neoplasms with hyperandrogenemia: a retrospective analysis of 33 cases.

The aim of this study is to review the natural course, clinical features, and reproductive prognosis of ovarian tumors associated with hyperandrogenemia. We retrospect 33 patients of ovarian tumors with hyperandrogenemia. Thirty cases (91%) were sex cord-stromal tumors. Sertoli-Leydig cell tumors, Leydig cell tumors, and steroid cell tumors were the most common types. It is not possible, to predict the pathological subtypes based on androgen levels alone. Most of these tumors were solid masses, with an average diameter of 3.9 cm. These tumors are soft or fragile, no clear boundary with normal tissue, thus excision is superior to exfoliation. The average disease course of the top three tumors was 32.6, 35.4, and 67.7 months, respectively. Among 11 married women with a desire to get pregnant, nine cases resumed menstrual periods after surgery and became pregnant naturally. Hyperandrogenemia might predict a better prognosis. The asynchronism of hyperandrogenemia and undetectable tumor may cause irreversible change and emotional depress, the methods of early diagnosis need further study.

An 88-year-old woman with flushing, alopecia and hirsutism and a Sertoli-Leydig cell tumour.

Sertoli-Leydig cell tumour (SLCT) is a rare, androgen-secreting sex cord-stromal tumour of the ovary that usually occurs in young premenopausal women. The major clinical manifestations are virilisation and defeminisation. The following case describes an 88-year-old G1P1 woman, 40 years after menopause, who presented with flushing, hirsutism, voice changes and alopecia along with significantly elevated levels of testosterone. Postoperative report revealed a well-differentiated SLCT in the left ovary. This case is unique in that SLCT is a very rare cancer and even more so in an 88-year-old woman. Taking this case into consideration, it becomes reasonable to check androgen and oestrogen levels in postmenopausal women, not only in patients with signs of virilisation, but also in those with non-classical presentations, such as flushing or heat spells.

Case report: an identical twin with Sertoli-Leydig cell tumor.

Our report details the workup and management of a 43-year-old woman with an identical twin who presented with 2 years of virilization and secondary amenorrhea. Serum total testosterone was elevated. An MRI did not identify adnexal or adrenal pathology. Subsequent ovarian vein sampling demonstrated unilateral testosterone elevation. The patient underwent laparoscopic unilateral oophorectomy resulting in the diagnosis of Sertoli-Leydig cell tumor (SLCT). Although SLCT is a rare sex-cord ovarian tumor, it is associated with endometrial hyperplasia and malignancy. Our goals are to review the workup of androgen-secreting tumors and discuss the clinical importance of the DICER1 mutation in the context of SLCT. In this case, an identical twin underwent DICER1 testing which was one of the essential steps in her clinical management.

Ovarian Sertoli-Leydig Cell Tumor with Elevated Inhibin B as a Cause of Secondary Amenorrhea in an Adolescent with Germ Line DICER1 Mutation.

BACKGROUND: Ovarian tumors, although uncommon in children, can retain endocrine function that disrupts normal feedback mechanisms leading to amenorrhea. Inheritance of germline DICER1 mutations can lead to increased risk for development of ovarian Sertoli-Leydig cell tumors (SLCTs). CASE: We report, to our knowledge, the first case of secondary amenorrhea due to elevated inhibin B levels in a female adolescent with an ovarian SLCT. SUMMARY AND CONCLUSION: Ovarian tumors should be included in the differential diagnosis for pediatric patients who present with menstrual irregularities. Early evaluation of the hypothalamic-pituitary-ovarian axis and inhibin levels is appropriate. Our case also emphasizes the need for testing for DICER1 mutations in pediatric patients with ovarian SLCTs.

Tumores virilizantes del ovario: presentación de dos casos manejados mediante cirugía por monopuerto / Virilizing tumors of the ovary: presentation of two cases managed by single-port surgery

Los tumores virilizantes, corresponden al 1% de todos los tumores funcionales del ovario. Estos tipos de tumores virilizantes se originan de las células pluri-potenciales del estroma ovárico, tienen la capacidad de secretar 17-hidroxiprogesterona, testosterona y androstenediona, desencadenando hiperandrogenismo clínico. Son catalogados como de bajo potencial maligno, con un patrón de crecimiento lento, bien diferenciados, diagnosticados en su mayoría en estadío I y II, de buen pronóstico y típicos de mujeres en edad reproductiva. El objetivo de esta comunicación es presentar dos casos clínicos con diagnóstico de tumor virilizante de ovario, tratadas con cirugía laparoscópica por mono puerto.

Virilizing tumors, corresponding to 1% of all functional ovarian tumors. Those type of virilizing tumors originate from pluripotential ovarian stromal cells and have the capacity to secrete 17-hydroxyprogesterone, testosterone and androstenedione, triggering clinical hyperandrogenism. They are classified as low malignant potential, well differentiated, with a pattern of slow growth, mostly diagnosed in stage I and II, with good prognosis and typical of women of reproductive age. The aim of this paper is to present two cases of virilizing ovarian tumor treated by mono port laparoscopic surgery.

Sertoli-Leydig cell tumors: hormonal profile after dynamic test with GnRH analogue: triptorelin represents a useful tool to evaluate tumoral hyperandrogenism.

We report the case of a 15-year-old woman with signs of hyperandrogenism affected by a Sertoli-Leydig cell tumor (SLCT). In our patient, blood analysis showed a high testosterone (T) level (T: 8.53 nmol/L; nv < 1.87 nmol/L) while the GnRH-analogue test demonstrated an exaggerated secretion of 17-hydroxyprogesterone (OHP), T, and androstenedione (A) by the ovary after stimulation. We compared the GnRH-analogue test of our patient with that obtained in a group of normal and healthy women (no. 8 subjects, 16-26 years old), men (no. 4 subjects, 18-28 years old), and in a group of PCOS patients with age and body weight compared. We found in our patient a value of OHP, 17-beta estradiol (E2) and T, from 2 to 18 times higher than healthy women. When we compared our patient with healthy men, we differently observed a comparable response of T. The response of our patient was also comparable with that observed in the PCOS group for E2. During the post-surgical follow up, the GnRH-analogue test of our patient showed a response of OHP, T, and E2 comparable with that of the PCOS group. The GnRH-analogue test is a useful tool to characterize steroidogenesis in SLCT.

Complete androgen insensitivity syndrome with a Sertoli-Leydig cell tumor.

BACKGROUND: The complete androgen insensitivity (testicular feminization) syndrome was described in phenotypic females with 46XY karyotype, presenting with primary amenorrhea, adequate breast development, and absent or sparse pubic or axillary hair. Gonads consist usually of seminiferous tubules without spermatogenesis. CASE: We report the case of a 15-year-old girl with testicular feminization since age 4. She was admitted to surgically remove the testes from the inguinal canal. Microscopic examination of the left testis revealed a Sertoli-Leydig cell tumor and the draft of fallopian tube with adjacent seminiferous tubules. SUMMARY AND CONCLUSION: These patients have a 5%-10% risk of developing germ cell tumors. Sertoli-Leydig cell tumors are uncommon neoplasms, usually benign in testicular feminization syndrome.

No metabolic impact of surgical normalization of hyperandrogenism in postmenopausal women with ovarian androgen-secreting tumours.

AIM: To examine the impact of surgical normalization of testosterone on body weight and on glucose and lipid metabolism and insulin sensitivity in a group of hyperandrogenic women with ovarian androgen-secreting tumours (OAST). METHODS: Five consecutive postmenopausal hyperandrogenic patients (aged 63 ± 5 years) with a diagnosis of OAST were prospectively evaluated. Clinical signs, symptoms and metabolic and hormonal parameters were collected at the time of the diagnosis and at follow-up, 12 months after surgical oophorectomy. A group of 15 age-matched and body mass index-matched postmenopausal control women served as a reference group. RESULTS: At baseline, patients with OAST had very high testosterone levels and inappropriately low gonadotrophin levels for their menopausal status. All the women were overweight or obese, and one had a history of polycystic ovary syndrome and Type 2 diabetes. Twelve months after surgical oophorectomy, testosterone and gonadotrophin levels returned to appropriate values for menopausal status in all patients; however, no change in body weight was found. Fasting glucose levels slightly increased (P < 0·05) without any significant change in other metabolic parameters. In the woman with diabetes, a moderate decrease in haemoglobin A1c occurred. Red blood cell count and haematocrit values were normalized (P < 0·05, respectively). CONCLUSION: Normalization of androgen levels achieved after surgical oophorectomy did not cause any significant change in body weight and insulin sensitivity. These findings may offer a different perspective on the impact of hyperandrogenaemia on metabolism.

Malignant ovarian Sertoli-Leydig cell tumor localized with selective ovarian vein sampling.

Sertoli-Leydig cell tumors (SLCT) are rare, comprising less than 0.5% of ovarian neoplasms. They are most often diagnosed in premenopausal women and may produce androgens, resulting in hirsuitism, voice deepening, frontal balding, terminal hair growth, and clitoromegaly. SLCT are malignant in 15%-20% of cases. We discuss a 25-year-old patient with persistent hyperandrogenemia. Noninvasive imaging cannot conclusively differentiate between SCLT and other diagnoses such as polycystic ovary syndrome, ovarian hyperthecosis, idiopathic hyperandrogenism, idiopathic hirsuitism, and 21-hydroxylase-deficient nonclassic adrenal hyperplasia. Selective ovarian vein sampling revealed a 15-fold greater testosterone production from the right ovary compared with the left, which guided appropriate surgical management.

Sertoli-Leydig cell tumor of the ovary--morphological and immunohistochemical analysis.

Sertoli-Leydig cell tumor is a rare and usually unilateral tumor of the ovary occurring in women's reproductive age. Only about 10% of these patients are over 50 years of age. One third of these patients are suffering from signs of virilisation. This work summarizes the morphological and immunohistochemical characteristics of this tumor in a 56-year old woman with clinical signs of virilisation.

Bilateral androblastoma (Sertoli-stromal cell tumor) of the ovary: a rare cause of virilization in a teenager.

A case of a 17-year-old patient diagnosed with bilateral androblastoma of the ovary is presented. The patient was admitted because of secondary amenorrhea, hirsutism and acne. After clinical, ultrasonographic and hormonal examinations an androgen-producing ovarian tumor was suspected and consequently laparotomy with right ovarian tumor excision and left ovary exploration was carried out. During surgery the right ovarian tumor was excised and exploration of the left ovary revealed an ovarian tumor with a diameter of 10 mm, which was then also excised. The pathologic diagnosis was a bilateral androblastoma of the ovary measuring 40 mm x 30 mm x 20 mm in the right ovary and 10 mm in diameter in the left ovary. We concluded that androblastomas, in spite of their low incidence, are a possibility that should always be considered in women of all ages presenting with signs of virilization.

[Malignant ovarian tumour as cause of virilisation in a 12 year-old girl]. / Malign ovarietumor som årsag til virilisering af en 12-årig pige.

Virilisation in girls includes androgen dependent growth of terminal hair, acne, deepening of the voice and increased muscle mass. Sudden onset of symptoms, rapid progression, short duration and hyperandrogenaemia are ominous signs and the patient must be referred to a specialist as soon as possible to rule out cancer and avoid worsening of the irreversible virilisation. We describe a girl with severe virilisation as the sole symptom of an androgen producing Sertoli-Leydig cell tumour. Ovarian tumours are rare in children and malignant ovarian tumours account for less than 1% of childhood cancer.

[Malignant arrhenoblastoma. Case report and literature review]. / Arrenoblastoma maligno. Caso clínico y revisión bibliográbfica.

The arrenoblastome is an ovary tumor with masculine hormone production, testosterone and other hormones. Other names are: stromatic tumor or gonadal stromatic tumor, also steroid cell tumor. They are rare tumors; represent 0.5% of all ovary tumors. It could be present in all age women groups, more frequently in young people. Most of times unilateral (95%), solids or quistic-solids. Anaplastic grade give them a malignity disease in 5 to 10 % cases. We report the case of a 35 year-old woman with clinical appearance of androgenism for ovary tumor, she was accepted for surgery, founded 7 liters of ascitis, produced for an ovary tumor, integral capsule, it produced masculine hormones. Histological study reported ovarian sex cord tumor, high grade, 30 cm size, integral capsule, all normally. Stage IC. Size and differential cellular grade need systemic chemotherapy. At the time of this report her tumoral marks are normal, and she has gradual diminution of virilizing characters produced for ovary tumor. Prognosis of the disease depends the grade of cell differentiation and stage in surgical-pathological events. Survival to five years stage I is approximate in 70 to 90% of the cases. Angular stone treatment is surgery. Disseminate cases, chemotherapy or radiotherapy most be considerate. Usually arrenoblastome has poor possibilities of dissemination and considering the early detection the histological grade of healthy is very high.