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 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)

Tumor de células de Leydig, presentación con pubertad precoz

Objetivos: Presentar un caso de pubertad precoz secundaria a tumor de células de Leydig. Caso Clínico: Paciente preescolar masculino de 5 años, quien fue referido por presentar aparición de vello púbico, aumento de tamaño del pene, piel oleosa y crecimiento acelerado para la edad, sin antecedentes de traumatismo craneal o procesos infecciosos cerebrales. Al examen físico: peso 22,7kg y talla 117,2 cm, ambos en el percentil 97, masas musculares evidentes en tórax y miembros superiores, vello púbico tanner II, pene de 6cm de longitud, con volumen testicular de 4 mL el derecho y de 3 mL el izquierdo, observándose mucho adelanto de caracteres sexuales secundarios para el volumen testicular. Los estudios de laboratorio revelaron valores elevados de andrógenos: testosterona libre: 237 ng/dL, 17OHP: 3.7 ng/mL, prueba de GnRH con respuesta prepuberal, no plana (pico de LH: 3,4 y de FSH: 1,8 ng/mL), prueba de estimulación con ACTH reporta 17OHP basal 5.3 y postestímulo 6 ng/mL, TSH: 2,1 mUI/mL, T4L: 1,10 ng/dL. Edad ósea de 10 años, relación EO/EC de 1.9, predicción de talla final de 157 cm, con potencial genético de talla de 169,7. Se planteó Pubertad Precoz Periférica (HAC hiperplasia adonal congénita vs. Gonadal) que desencadenó una pubertad verdadera. Se indicó tratamiento con Triptorelina e Hidrocortisona a dosis habituales por kg de peso. En su evolución clínica, a pesar del tratamiento y mostrando normalización de niveles de 17OHP (1.4 ng/mL) y adecuada supresión del eje Hipotálamo-Hipófisis-Gónada, continuó progresión de caracteres sexuales secundarios, aumentando la asimetría testicular VD: 8mL y VI: 6mL, pene de 9 cm y la velocidad de crecimiento de 12 cm/año. Nuevos niveles de testosterona muy elevados (770 ng/dL). TAC de abdomen normal y ultrasonido testicular reportó LOE sólido en testículo derecho. Marcadores tumorales normales, excepto ligera elevación de Gonadotropina Coriónica. Se realiza orquidectomía derecha y ligadura alta de cordón. El estudio anatomopatológico reportó tumor de células de Leydig sin signos de malignidad. Conclusiones: Los tumores testiculares son muy raros en niños (1 a 2%) y aproximadamente el 1 a 3% de éstos, corresponden a los de células de Leydig, que se presentan con desarrollo somático precoz y virilización progresiva, siendo una causa de precocidad puberal.

Objectives: To report a case of precocious puberty due to a Leydig cell tumor. Clinical Case: A 5 years old male patient, with pubic hair, penis enlargement, oiliness of skin and accelerated growth was referred. There was no previous cranial traumatism or cerebral disease. Physical examination: weight 22,7 kg, height 117,2 cm, both over 97th percentile for age, muscular development in thorax and upper extremities, pubic hair (Tanner II), penis of 6 cm of longitude, volume of right testicle 4 mL and left 3 mL. More signs of pubertal development than would be expected for the size of the testis were evident. The laboratory analysis showed elevated levels of androgens: free testosterone: 237 ng/dL, 17OHP: 3,7 and 5,3 ng/mL basally and 6 ng/mL post stimulation with ACTH, slight response to the GnRH stimulation test (LH peak: 3,4 and FSH peak: 1,8 ng/mL), normal levels of TSH and FT4. Bone age of 10 years, BA/CA of 1,9, predicted adult height of 157 and target height of 169,7 cm. The diagnostic of peripheral precocious puberty (congenital adrenal hyperplasia vs gonadal tumor) that triggered a central precocity was suggested. Treatment with Triptorelin and Hydrocortisone was initiated in usual doses. With this therapy, the 17OHP levels were normal and a suppression of LH and FSH were obtained. Nevertheless, the boy showed progression of the pubertal development, right testicle of 8 mL, left of 6 mL, penis of 9 cm and growth velocity of 12 cm/ year. Testosterone levels were higher (770 ng/dL). Abdominal computerized axial tomography was normal and the testicular ultrasonography disclosed a solid tumor in the right testicle. Tumor markers were normal. Surgical removal of the right testicle was done. The histopathology study revealed a Leydig cell tumor without malignancy signs. Conclusions: Testicular tumors are rare in children (1 a 2%) and 1-3% of them are Leydig cell tumors. They may cause incomplete precocious puberty with somatic development and progressive virilization.

Tumor de células de leydig em crianças - relato de caso / Leydig cell tumor in child - case report

Objetivo: relatar um caso em criança de sete anos, apresentando puberdade precoce e submetida à orquiectomia total esquerda por via inguinal, devido dificuldade no diagnóstico preciso do tumor de células de Leydig, durante o ato cirúrgico. Considerações finais: as manifestações endócrinas devem ser levadas em consideração para o diagnóstico diferencial, fazendo com que crianças possam, efetivamente, se beneficiar de cirurgias menos radicais. Após um ano de seguimento não houve recidiva clínica ou hormonal do tumor.

Case Report: the authors report a case of a seven-year-old child, with isosexual precocity, in who had performed a left radical inguinal orchiectomy, due to difficulties in precise diagnosis during surgery. Final Considerations: manifestations of endocrine imbalance could help in diagnosis, permitting further children benefit least radical procedures. No clinical or endocrine relapse after 1 year follow-up.