Carcinoma adrenal virilizante en una adolescente: reporte de caso / Virilizing adrenal carcinoma in an adolescent: case report

Los tumores suprarrenales virilizante son infrecuentes y representan 5-6% de los tumores de esas glándulas1. Pueden secretar diferentes andrógenos como dehidroepiandrosterona sulfato (DHEAS), androstenediona y testosterona. Las características clínicas dependen de la edad de presentación; en niños pueden determinar pubertad precoz y en mujeres en edad fértil ocasionar hirsutismo, amenorrea o ciclos oligomenorreicos y diversos grados de virilización2. Los carcinomas adrenocorticales son tumores raros y la incidencia es aproximadamente uno a dos por millón de habitantes/año3,4. Los exámenes de imagen como la tomografía o la resonancia confirman el origen suprarrenal, valoran la presencia de metástasis y definen la conducta terapéutica5. La presentación inicial en pacientes pediátricos mayoritariamente es con virilización6 y aproximadamente el 50% de los pacientes adultos con carcinoma adrenal tienen un estadio de la enfermedad relativamente avanzado7. El tratamiento de elección es la cirugía y sigue siendo la mejor esperanza para la supervivencia a largo plazo8. El pronóstico habitual para el carcinoma adrenocortical es generalmente malo con una supervivencia global a 5 años de 20 a 25%5 en adultos, pero en niños y adolescentes la supervivencia puede llegar al 100%9. Se presenta el caso de una paciente con fenotipo totalmente masculino con diagnóstico de carcinoma adrenal virilizante que comienza en la infancia y se diagnostica en la adolescencia.

Virilizing adrenal tumors are uncommon and represent 5-6% on tumors of these glands1. They can secrete different androgens such as dehydroepiandrosterone sulfate (DHEAS), androstenedione, and testosterone. Clinical features depend on the age of presentation; in children they can determine precocious puberty and in women of childbearing age cause hirsutism, amenorrhea or oligomenorrheic cycles and various degrees of virilization2. Diagnosis consists of clinical evidence of hyperandrogenism, accompanied by an increase in androgens in the blood, especially DHEAS, whose origin is mainly adrenal. Adrenocortical carcinomas are rare and the incidence is approximately one to two per million inhabitants/year3,4. Imaging tests such as tomography or resonance confirm the adrenal origin, assess the presence of metastases and define the therapeutic approach5. In initial presentation in most pediatric patients is with virilization6 and approximately 50% adult's patients with adrenal carcinoma have a relatively advanced stage of the disease7. The treatment of choice is surgery and is the best hope for long-term survival. The usual prognosis for adrenocortical carcinoma is generally poor with a 5-year overall survival of 20 to 25%5 in adults, but in children and adolescent's survival can reach 100%9. We present the case of a patient with a totally male phenotype diagnosed with virilizing adrenal carcinoma that begins in childhood and is diagnosed in adolescence.

Tumor virilizante gigante de células de la granulosa de ovario izquierdo. Caso clínico / Giant virilizing tumor of left ovarian granulose cells. Clinical case / Tumor virilizador gigante de células de granulose ovariana esquerda. Caso clínico

RESUMEN Los tumores productores de esteroides ováricos constituyen entidades infrecuentes y son potencialmente malignos. La testosterona es la hormona que se eleva con más frecuencia y se asocia en su mayoría a signos de virilización. Se diagnosticó un caso de una paciente de 62 años con tumor gigante virilizante de células de la granulosa de ovario. Se realizo laparotomía programada con biopsia por congelación e histerectomía total abdominal con doble anixectomía más omentectomía. Evoluciono favorablemente sin complicaciones.El caso que aquí se reporta es el de una paciente con antecedentes de salud aparente, menstruaciones regulares, con antecedentes obstétricos de 4 gestaciones y de haber sido operada a los 24 años de un óbito fetal de mellizos, ante un cuadro de síndrome de virilización se utilizaron los datos provenientes de la historia clínica hospitalaria, exámenes complementarios, la entrevista y el examen físico de la paciente. A pesar del tiempo de evolución que presentaba la paciente con el tumor virilizante gigante de células de la granulosa de ovario izquierdo, la operación fue un éxito y la evolución ha sido satisfactoria, gracias a la labor del personal médico que estuvo a cargo del caso, demostrando una vez más las fortalezas del sistema cubano de salud ante situaciones como estas.

ABSTRACT Peripheral steroid-producing tumors are rare entities and are potentially malignant. Testosterone is the most commonly elevated hormone and is mostly associated with signs of virilization. A case of a 62-year-old patient with a giant virilizing ovarian granulose cell tumor was diagnosed. Scheduled laparotomy was performed with freezing biopsy and total abdominal hysterectomy with double anixectomy plus omentectomy. I evolve favorably without complications. The case reported here is that of a patient with a history of apparent health, regular menstruations, with an obstetric history of 4 gestations and having been operated on at 24 years of a fetal heart rate, before a table of virilization syndrome were used data from hospital medical history, complementary examinations, interview and physical examination of the patient. Despite the time of evolution presented by the patient with the giant virilizing tumor of cells of the left ovary granulose, the operation was a success and the evolution has been satisfactory, thanks to the work of the medical staff who were in charge of the case, demonstrating once again the strengths of the Cuban health system in situations like these.

RESUMO Tumores periféricos produtores de esteroides são entidades raras e são potencialmente malignos. A testosterona é o hormônio mais comumente elevado e está principalmente associado com sinais de virilização. Um caso de um paciente de 62 anos com um tumor gigante de células de granulose ovariana foi diagnosticado. A laparotomia programada foi realizada com biópsia congelante e histerectomia abdominal total com anixectomia dupla mais omentectomia. Eu evoluo favoravelmente sem complicações. O caso aqui relatado é o de um paciente com histórico de saúde aparente, menstruações regulares, com histórico obstétrico de 4 gestações e tendo sido operado aos 24 anos de frequência cardíaca fetal, antes de uma tabela de síndrome de virilização ser utilizado dados do histórico médico hospitalar, exames complementares, entrevista e exame físico do paciente. Apesar do tempo de evolução apresentado pelo paciente com o tumor virilizador gigante de células do ovário esquerdo granulose, a operação foi um sucesso e a evolução tem sido satisfatória, graças ao trabalho da equipe médica responsável pelo caso, demonstrando mais uma vez os pontos fortes do sistema de saúde cubano em situações como essas.

Sex cord stromal tumor of ovary masquerading as polycystic ovarian syndrome

Virilization is a portentous sign that suggests the possibility of an ovarian or adrenal neoplasm. Diagnosis may be delayed in some patients due to nonspecific symptoms and overlapping symptoms with that of polycystic ovarian syndrome (PCOS). However, it must be remembered that PCOS usually causes mild to moderate elevation of serum testosterone with hirsutism whereas serum testosterone levels are many times elevated in cases of androgen secreting tumors and virilization is a norm. So high testosterone level with new onset virilization rule out PCOS. Authors are reporting two cases of Sertoli Leydig cell tumor despite their similar histopathology and equivalent levels of serum testosterone had a varied clinical spectrum of virilization.

Tumor de células de Leydig puro de ovario en mujer premenopáusica / Pure Leydig cell tumor of the ovary in a premenopausal woman / Pure Leydig cell tumor of the ovary in a premenopausal woman

RESUMEN Los tumores de células de Leydig del ovario son un tipo raro de tumores del estroma del cordón sexual, con menos de 0,1% de todos los tumores de ovario. Representan un desafío diagnóstico, no solo por su incidencia esporádica sino también por presentar imágenes aparentemente normales. Aunque son más comunes en las mujeres menopaúsicas, también se ha descrito casos en mujeres premenopáusicas. La característica clínica más común es la aparición de virilización rápida y progresiva; más de 75% de las pacientes muestra signos de virilización debido a la sobreproducción de testosterona. La concentración sérica de testosterona representa el marcador más útil en el diagnóstico del tumor ovárico secretor de andrógenos. El tumor de células de Leydig ovárico siempre debe ser considerado en mujer en edad reproductiva con síntomas de virilización. Se presenta un caso de tumor de células de Leydig puro de ovario en mujer premenopáusica.

ABSTRACT Leydig cell tumors of the ovary are a rare type of sex cord-stromal tumors, corresponding to less than 0.1% of all ovarian neoplasms. With a low incidence and frequent false-negative imaging results, these tumors represent a diagnostic challenge. Although more common in menopause, cases have also been described in premenopausal women. The most common clinical feature is rapidly progressive virilization; over 75% of patients show signs of virilization due to testosterone overproduction. Serum testosterone concentration is the most useful marker for diagnosing androgen-secreting tumors of the ovary. Leydig cell tumors should always be considered in women of reproductive age with virilization symptoms. We present the case of a pure Leydig cell tumor of the ovary in a premenopausal woman.

Tumor ovárico virilizante en mujer postmenopáusica: reporte de caso / Ovarian virilizing tumor in postmenopausal woman: case report

Ovarian steroid-producing tumors are infrequent entities and are potentially malignant. Testosterone is the hormone that rises more frequently and is associated mostly with signs of virilization. We present the clinical case of a 67-year-old postmenopausal woman who came to the clinic for alopecia, with high levels of testosterone and ovarian mass by ultrasound. Surgical treatment was indicated. The main diagnostic aspects are presented.

Los tumores productores de esteroides ováricos constituyen entidades infrecuentes y son potencialmente malignos. La testosterona es la hormona que se eleva con más frecuencia y se asocia en su mayoría a signos de virilización. Se presenta el caso clínico de una mujer postmenopáusica de 67 años que acude a consulta por alopecia, con niveles elevados de testosterona y masa ovárica por ecografía. Se indicó tratamiento quirúrgico. Se presentan los principales aspectos diagnósticos.

Testosterone undecanoate supplementation together with human chorionic gonadotropin does not impair spermatogenesis in males with isolated hypogonadotropic hypogonadism: A retrospective study / 亚洲男科学杂志(英文版)

Gonadotropin therapy is commonly used to induce virilization and spermatogenesis in male isolated hypogonadotropic hypogonadism (IHH) patients. In clinical practice, 5.6%-15.0% of male IHH patients show poor responses to gonadotropin treatment; therefore, testosterone (T) supplementation can serve as an alternative therapy to normalize serum T levels and promote virilization. However, treatment with exogenous T impairs spermatogenesis and suppresses intratesticular T levels. This retrospective study aimed to determine whether oral testosterone undecanoate (TU) supplementation together with human chorionic gonadotropin (hCG) would negatively affect spermatogenesis in IHH patients compared with hCG alone. One hundred and seven IHH patients were included in our study. Fifty-four patients received intramuscular hCG and oral TU, and 53 patients received intramuscular hCG alone. The median follow-up time was 29 (range: 12-72) months in both groups. Compared with the hCG group, the hCG/TU group required a shorter median time to normalize serum T levels (P < 0.001) and achieve Tanner stage (III and V) of pubic hair and genital development (P < 0.05). However, there were no significant differences in the rate of seminal spermatozoa appearance, sperm concentration, or median time to achieve different sperm concentration thresholds between the groups. In addition, there were no significant differences in side effects, such as acne and gynecomastia, observed in both groups. This study indicates that oral TU supplementation together with hCG does not impair spermatogenesis in treated IHH patients compared with hCG alone, and it shortens the time to normalize serum T levels and promote virilization.

Carcinoma adrenocortical oncocítico virilizante / Adrenal oncocytic virilizing carcinoma

Las neoplasias oncocíticas se encuentran raramente en la glándula suprarrenal. Suelen ser benignos y no funcionales. Existen informes extremadamente limitados de neoplasias oncocíticas suprarrenales. Alrededor del 20% de las neoplasias oncocíticas adrenocorticales muestran componentes malignos y el 10-20% produce hormonas que pueden causar desbalances hormonales. Se presenta un carcinoma adrenocortical oncocítico virilizante en mujer de 35 años de edad quien acudió a consulta por presentar hipomenorrea secundaria de seis meses de duración acompañada de hirsutismo y agravamiento del acné que afectaba principalmente la cara. El examen de laboratorio revela niveles elevados de testosterona libre. La tomografía computada mostró tumor multinodular en la glándula suprarrenal derecha con necrosis central y sin evidencia de linfadenopatía o invasión de estructuras que la rodeaban. Se programó la adrenalectomía derecha lográndose resección completa del tumor. Después de la cirugía, se normalizaron los ciclos menstruales, el hirsutismo, el acné y el resto de las pruebas. El examen histopatológico del tumor fue compatible con carcinoma adrenocortical oncocítico(AU)

Oncocytic neoplasms are found more rarely in the adrenal gland. They are usually benign and nonfunctional. There are limited reports of adrenal oncocytic neoplasms. About 20% of adrenocortical oncocytic neoplasms show malignant components and 10-20% produce hormones that can cause hormonal imbalances. We present a virilizing oncocytic adrenocortical carcinoma in a 35-year-old woman who consulted for hypomenorrhea accompanied by hirsutism and face acne worsening. Laboratory test revealed high levels of free testosterone. Computed tomography showed multinodular tumor of the right adrenal gland with central necrosis and without evidence of lymphadenopathy or invasion of surrounding structures. A right adrenalectomy was performed. After surgery, menstrual cycle, hirsutism and acne, as well as hormonal tests were normal. Histopathological examination of tumor showed an oncocytic adrenocortical carcinoma(AU)

CLITOROPLASTY FOR GENITAL VIRILIZATION IN GIRLS WITH CONGENITAL ADRENAL HYPERPLASIA / 中国修复重建外科杂志

OBJECTIVE: To investigate the effectiveness of clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate for genital virilization in girls with congenital adrenal hyperplasia (CAH). METHODS: Between June 2000 and October 2015, 27 girls with genital virilization were treated with clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate. The chromosomal karyotype was 46, XX in all patients, whose age ranged from 3 to 11 years (mean, 3.9 years). According to Parder's virilization scale, 5 cases were classified as stage Ⅱ, 18 cases as stage Ⅲ, and 4 cases as stage Ⅳ. The hormone tests showed decreased cortisol level and increased testosterone and 17-hydroxyprogesterone levels. They were diagnosed with non-salt-wasting CAH. RESULTS: All of the patients underwent clitoroplasty successfully. The mean operation time was 74 minutes (range, 58-95 minutes). Incision healed primarily. The patients were followed up 6 months to 10 years (median, 19 months). The external genitalia had good appearance, without necrosis of flap and the glans clitoris, abnormal sensation of the glans clitoris, or clitorism recurrence. Five girls had breast development and normal menstrual cycle during adolescence, and 1 patient who was followed up for 10 years gave birth to a son. CONCLUSIONS: Clitoroplasty of reserving the clitoris dorsal neurovascular bundle and the ventral complete urethral plate is a relatively ideal method for treating genital virilization in girls. It has less complications, good aesthetic and functional results. There are satisfactory outcomes when combining with the endocrine treatment.

Tumor de células de Leydig do ovário associado a virilização em paciente na pós-menopausa / Ovarian Leydig cell tumor associated with virilization in postmenopausal patient

Tumores de células de Leydig são neoplasias de células esteroides e correspondem a menos de 0,5% dos tumores ovarianos. Ocorrem mais comumente na pós-menopausa e se apresentam com virilização em metade dos casos. Relatamos o caso de uma mulher de 53 anos com história de virilização. A investigação com ressonância magnética demonstrou altos níveis séricos de testosterona e um nódulo de 2 cm no ovário direito. A paciente foi submetida a ooforectomia bilateral, e a análise patológica confirmou o diagnóstico de tumor de células de Leydig do ovário direito. Um dia após a cirurgia, o nível sérico de testosterona se normalizou. Em quatro meses, a paciente apresentou nível sérico normal de testosterona e regressão parcial da alopecia. Em mulheres pós-menopáusicas com quadro de virilização progressiva, deve-se suspeitar de neoplasias ovarianas produtoras de andrógenos (AU)

Leydig cell tumors are tumors of the steroids cells and represent less than 0.5% of ovarian tumors. They occur most often in postmenopausal women and present with virilization in half of the cases. We report the case of a 53-year-old woman with virilization history. Magnetic resonance imaging showed high serum testosterone levels and a 2-cm nodule in the right ovary. The patient underwent bilateral oophorectomy, and the pathological analysis confirmed the diagnosis of Leydig cell tumor in the right ovary. The day after surgery, serum testosterone level was normalized. In four months, the patient had normal serum testosterone level and partial regression of alopecia. In postmenopausal women with progressive virilization, ovarian neoplasms producing androgens should be investigated (AU)

Leydig cell tumor of ovary with primary hyperparathyroidism: A rare association.

Leydig cell tumors of ovary are extremely rare sex cord stromal tumors that account for <0.2% of ovarian cancers. Most of these tumors can cause hyperandrogenism. A 54 year old multiparous postmenopausal woman, known diabetic and hypertensive, presented with progressive virilization, bone pains and depressed mood for 2 years. Diagnostic evaluation revealed markedly elevated testosterone with normal dehydroepiandrosterone sulphate. Computerized tomography of abdomen showed right ovarian mass with multiple cysts in both kidneys. Biochemical investigations revealed hyperglycemia, hypercalcemia, hypophosphatemia and elevated parathormone (PTH) levels. Sestamibi scan for parathyroids was suggestive of right inferior parathyroid adenoma. Histopathology of the resected right ovarian tumor was consistent with leydig cell tumor of ovary. Postoperatively serum testosterone and blood glucose levels were normalized, but PTH levels remained high. PTH levels were normalized after resection of parathyroid adenoma

Manejo de la hiperplasia suprarrenal congénita

La hiperplasia adrenal congénita (HSC) es un grupo de desórdenes causados por defectos en la esteroidogénesis adrenal. En su forma más común, la deficiencia de la enzima 21-α-hidroxilasa, los pacientes desarrollan grados variables de deficiencia de glucocorticoides y mineralocorticoides, así como de exceso de andrógenos. En general se clasifica en formas clásicas y no clásicas. Los objetivos del tratamiento son sustituir la deficiencia de cortisol y aldosterona, evitar el hiperandrogenismo y lograr la mejor talla final; aunque estos objetivos parecen bastante sencillos, en la práctica, son muy difíciles de lograr. La terapia con glucocorticoides y mineralocorticoides es guiada por el monitoreo de variables clínicas y de concentraciones de hormonas androgénicas y electrolitos. Con respecto al crecimiento, se debe mantener un delicado equilibrio; el sobretratamiento con glucocorticoides puede conducir a deterioro del mismo, y un tratamiento insuficiente, al exceso de andrógenos y maduración epifisaria prematura. Se presenta el protocolo de diagnóstico y tratamiento de la HSC de la Unidad de Endocrinología del Instituto Autónomo Hospital Universitario de Los Andes, Mérida, Venezuela.

Congenital adrenal hyperplasia (CAH) is a group of disorders caused by defects in the adrenal steroidogenic pathways. In its most common form, 21-α-hydroxylase deficiency, patients develop varying degrees of glucocorticoid and mineralocorticoid deficiency as well as androgen excess. It is generally classified as classical and non-classical forms. The goals of treatment are to replace the cortisol and aldosterone deficiency, avoid hyperandrogenism and achieve the best final height. Although these goals seem pretty straightforward, in practice, they are very difficult to achieve. Glucocorticoid and mineralocorticoid therapy is guided by monitoring clinical parameters as well as adrenal hormone and electrolytes concentrations. Practitioners must strike a fine balance; on height, overtreatment with glucocorticoids can lead to poor growth, and undertreatment, to androgen excess and premature epiphyseal maturation. The CAH diagnosis and treatment of the Endocrinology Service, Autonomous Institute University Hospital of The Andes, Mérida, Venezuela.

Androgen - secreting steroid cell tumor of the ovary.

Steroid cell tumors (SCTs), not otherwise specifi ed of the ovary are rare subgroup of sex cord tumors, which account for less than 0.1% of all ovarian tumors and also that will present at any age. The majority of these tumors produce steroids with testosterone being the most common. A case of a 28-year-old woman who presented with symptoms of virilization is reported. Although SCTs are generally benign, there is a risk for malignant transformation. Surgery is the most important and hallmark treatment.

Female sexual abnormality caused by androgen-producing adrenocortical adenoma：One case report / 中华内分泌代谢杂志

Objective To describe a case of female sexual abnormality with 46, XX caused by an androgen-producing adrenocortical tumor and to explore the mechanism of abnormal androgen secretion from the tumor. Methods The tumor tissues as the experimental group were compared with the normal adrenal tissue. The LH/human chorionic gonadotropin ( hCG) receptor was determined by immunohistochemisty, the activity of 3β-hydroxysteroid dehydrogenase ( 3β-HSD ) , 17α-hydroxylase ( CYP17 ) , and 17β-hydroxysteroid oxidoreductase ( 17β-HSD ) by enzyme linked immunosorbent assay(ELISA) and the expression of mRNA of 3β-HSD2, 17β-HSDB3, CYP17, and LH/hCG receptor by real-quantitative polymerase chain reaction ( RQ-PCR ) . Results The immunohistochemisty results showed that the LH/hCG receptor was negative in the experiment group, but positive in control. The activity of 3β-HSD and CYP17 of the experiment group was higher than that in the control (P<0. 01), while the activity of 17β-HSD was lower(2 638. 798±70. 551 vs 9 148. 174±382. 836, P<0. 01) according to ELISA results. The relative contentof3β-HSD2mRNAoftheexperimentgroupwashigherthanthatinthecontrol(P<0.05),andtherelative content CYP17 mRNA of the experiment group was much higher than that in the control (P<0. 01). However, the relative content of 17β-HSDB3 mRNA and LH/hCG receptor mRNA were much lower than those in the control ( P<0. 01) by RQ-PCR. Conclusion Sexual abnormality and virilization could be caused by the excessive androgen secreted by androgen-producing adrenocortical tumor, which is an extremely rare disease. The mechanism of the secretion of androgen from the tumor remains unknown so far. It may be related to the increased activity of 3β-HSD and CYP17, but has no relationship with the expression of LH/hCG receptor.

Ovarian Stromal Tumors: A rare cause of postmenopausal virilization / Journal of the ASEAN Federation of Endocrine Societies

@#We report the case of a 61-year-old Filipino woman with postmenopausal virilization presenting with androgenic alopecia, progressive hirsutism and deepening voice over 5 years due to elevated testosterone levels. Ultrasound and computed tomography imaging suggested a left adnexal mass. Histology obtained following total abdominal hysterectomy and bilateral salpingo-oophorectomy (TAHBSO) demonstrated stromal luteoma of the left ovary. On immunohistochemistry, the tumor was positive for inhibin. Postoperatively, there was note of rapid normalization of testosterone level.

A Adrenocortical Oncocytoma in a 10-year-old Girl Presenting with Virilization / 임상소아혈액종양

Adrenocortical oncocytoma is a rare epithelial tumor only described in adults and in most cases, benign and non-functioning. Histologically characterized by cells with eosinophilic granular cytoplasm and ultra-structurally by the presence of numerous closely packed mitochondria. We report a case of adrenal oncocytoma developed in a 10-year-old girl who presented a left adrenal mass with low voice tone, excessive hair growth, and elevation of the plasma testosterone and dehydroepiandrosterone sulfate levels as a result of overproduction of adrenal steroid hormones. After the left adrenalectomy, her hormone levels were normalized.

A Adrenocortical Oncocytoma in a 10-year-old Girl Presenting with Virilization / 임상소아혈액종양

Adrenocortical oncocytoma is a rare epithelial tumor only described in adults and in most cases, benign and non-functioning. Histologically characterized by cells with eosinophilic granular cytoplasm and ultra-structurally by the presence of numerous closely packed mitochondria. We report a case of adrenal oncocytoma developed in a 10-year-old girl who presented a left adrenal mass with low voice tone, excessive hair growth, and elevation of the plasma testosterone and dehydroepiandrosterone sulfate levels as a result of overproduction of adrenal steroid hormones. After the left adrenalectomy, her hormone levels were normalized.

Tumor de células esteroideas de ovario: reporte de un caso y revisión de la literatura / Steroid cell tumor of the ovary: a case report and literature review

El tumor de células esteroideas de ovario es una neoplasia poco frecuente que se origina del estroma o de los cordones sexuales y es potencialmente maligno. La mayoría de estos tumores producen hormonas esteroides siendo la testosterona la que se eleva frecuentemente. Se presenta el caso de una paciente de 30 años que presentó signos de virilización asociado a masa pélvica, ascitis e incremento de la testosterona. Fue operada y sometida a estadiaje de ovario. El resultado del estudio anatomopatológico fue tumor de células esteroideas. Una revisión de los aspectos clínicos, diagnósticos y terapéuticos de estos tumores, es presentada.

Steroid cell tumor of the ovary is a rare malignancy originating from the stromal or sex cord and is potentially malignant. Most of these tumors produce steroid hormones testosterone being which arises frequently. We report a case of a patient 30 who showed signs of virilization associated with pelvic mass, ascites and increased testosterone. She was operated and subjected to ovarian cancer staging. The result of the pathology was steroid cell tumor. A review of clinical aspects, diagnostic, and treatment of these tumors, is presented.

An Ovarian Steroid Cell Tumor Causing Virilization and Massive Ascites

Steroid cell tumors, not otherwise specified (NOS), are rare ovarian sex cord-stromal tumors with malignant potential. The majority of these tumors produce several steroids, particularly testosterone. Various virilizing symptoms such as hirsutism, temporal balding, and amenorrhea are common in these patients; however massive ascites is an infrequent symptom. A 52-year-old woman with the sudden onset of virilization and massive ascites presented for treatment at Severance Hospital. After clinical evaluation, the patient underwent an exploratory laparotomy and a complete surgical staging procedure. She recovered from the surgery uneventfully and was discharged from the hospital five days after surgery. We present here an unusual case of an ovarian steroid cell tumor, NOS, and a brief review of the literature regarding these types of tumors.

Skin abnormality and hairloss: the reproductive endocrinological viewpoint.

Excessive androgen production may cause changes in female skin, such as hirsutism and acne. The administration of antiadrogenic hormone such as cyproteron acetate, may eliminate the hyperandrogenic effect on the skin. Hairloss may also caused either by hyper-androgenemia or by low estrogen level. The administration of either antiandrogen or estrogen may reduce hairloss. Virilization, which includes excessive growth of hair and clitoris enlargement, deepened voice, muscle hypertrophy and mammary hypoplasia are also associated with hyperandrogenemia. Antiandrogen treatment could eliminate these impacts of virilization. In contrast, cellulite was supected to be due to androgen deficiency, and the use of topical testosterone could eliminate it. It is concluded that skin and/or hairloss are associated with hormonal changes in women. The treatment with antiandrogenic hormones may reduce or cure these abnormalities.

Functioning Adrenocortical Carcinoma in a Child / 대한비뇨기과학회지

Adrenocortical carcinoma is a rare tumor in children. This tumor is more likely to be hormonally active in children than in adults and tends to cause a variety of symptoms. These tumors are usually diagnosed at the advanced stages and have a dismal prognosis. Here we report a case of a functioning adrenocortical carcinoma in a child with a review of the relevant literature.