RESUMO
BACKGROUND: Ovarian 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 PRESENTATION: We 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. CONCLUSION: The 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.
Assuntos
Neoplasias Ovarianas , Síndrome do Ovário Policístico , Tumores do Estroma Gonadal e dos Cordões Sexuais , Feminino , Humanos , Adolescente , Adulto Jovem , Adulto , Amenorreia/complicações , Síndrome do Ovário Policístico/complicações , Testosterona , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Neoplasias Ovarianas/cirurgia , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/diagnóstico , Tumores do Estroma Gonadal e dos Cordões Sexuais/cirurgia , Virilismo/etiologia , Virilismo/diagnósticoRESUMO
BACKGROUND: The study of neoplasia in wildlife species contributes to the understanding of cancer biology, management practices, and comparative pathology. Higher frequencies of neoplasms among captive non-domestic felids have been reported most commonly in aging individuals. However, testicular tumours have rarely been reported. This report describes a metastatic testicular sex cord-stromal tumour leading to fatal haemorrhage and thrombosis in a captive African lion (Panthera leo). CASE PRESENTATION: During necropsy of a 16-year-old male African lion, the left testicle and spermatic cord were found to be intra-abdominal (cryptorchid), semi-hard and grossly enlarged with multiple pale-yellow masses. Encapsulated haemorrhage was present in the retroperitoneum around the kidneys. Neoplastic thrombosis was found at the renal veins opening into the caudal vena cava. Metastases were observed in the lungs and mediastinal lymph nodes. Histology revealed a poorly differentiated pleomorphic neoplasm comprised of round to polygonal cells and scattered spindle cells with eosinophilic cytoplasm. An immunohistochemistry panel of inhibin-α, Ki-67, human placental alkaline phosphatase, cytokeratin AE1/AE3, cKit, vimentin and S100 was conducted. Positive cytoplasmic immunolabeling was obtained for vimentin and S100. CONCLUSIONS: The gross, microscopic and immunohistochemical findings of the neoplasm were compatible with a poorly differentiated pleomorphic sex cord-stromal tumour. Cause of death was hypovolemic shock from extensive retroperitoneal haemorrhage and neoplastic thrombosis may have contributed to the fatal outcome. To our knowledge, this is the first report of sex cord-stromal tumour in non-domestic felids.
Assuntos
Leões , Tumores do Estroma Gonadal e dos Cordões Sexuais/veterinária , Neoplasias Testiculares/veterinária , Animais , Evolução Fatal , Hemorragia/etiologia , Hemorragia/veterinária , Masculino , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologia , Neoplasias Testiculares/complicações , Neoplasias Testiculares/patologia , Trombose/etiologia , Trombose/veterináriaRESUMO
El tumor de los cordones sexuales con túbulos anulares es una neoplasia del estroma gonadal muy infrecuente. Representa el 0,05-0,6% de todos los tumores ováricos, según series. Se presenta un caso especialmente inusual, en una niña de 6 años, detectado a raíz de una pubertad precoz periférica isosexual. Su interés radica en que no se halló ninguna masa anexa al ovario, sino únicamente una asimetría gonadal, sin signos radiológicos de malignidad. Se realizó una salpingo-ooforectomía unilateral con linfadenectomía pélvica y paraaórtica ipsilateral, por vía laparoscópica, tras confirmarse la presencia de células tumorales en la biopsia intraoperatoria. La evolución posterior de la paciente fue favorable.
Sex cord tumor with annular tubules is an extremely uncommon gonadal stromal neoplasm. It represents 0.05-0.6% of all ovarian tumors, according to series. An unusual case is presented in a 6-year-old girl, detected as a result of an isosexual peripheral precocious puberty. The highlight of this case is that no mass attached to the ovary was found, but only a gonadal asymmetry without radiological signs of malignancy. After confirming the presence of tumoral cells by intraoperative biopsy, unilateral salpingo-oophorectomy with ipsilateral para-aortic and pelvic lymphadenectomy was performed. Afterwards, the evolution of the patient was favorable.
Assuntos
Humanos , Feminino , Criança , Neoplasias Ovarianas/complicações , Puberdade Precoce/etiologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Neoplasias Ovarianas/patologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologiaRESUMO
Sex cord tumor with annular tubules is an extremely uncommon gonadal stromal neoplasm. It represents 0.05-0.6% of all ovarian tumors, according to series. An unusual case is presented in a 6-year-old girl, detected as a result of an isosexual peripheral precocious puberty. The highlight of this case is that no mass attached to the ovary was found, but only a gonadal asymmetry without radiological signs of malignancy. After confirming the presence of tumoral cells by intraoperative biopsy, unilateral salpingo-oophorectomy with ipsilateral para-aortic and pelvic lymphadenectomy was performed. Afterwards, the evolution of the patient was favorable.
El tumor de los cordones sexuales con túbulos anulares es una neoplasia del estroma gonadal muy infrecuente. Representa el 0,05-0,6% de todos los tumores ováricos, según series. Se presenta un caso especialmente inusual, en una niña de 6 años, detectado a raíz de una pubertad precoz periférica isosexual. Su interés radica en que no se halló ninguna masa anexa al ovario, sino únicamente una asimetría gonadal, sin signos radiológicos de malignidad. Se realizó una salpingo-ooforectomía unilateral con linfadenectomía pélvica y paraaórtica ipsilateral, por vía laparoscópica, tras confirmarse la presencia de células tumorales en la biopsia intraoperatoria. La evolución posterior de la paciente fue favorable.
Assuntos
Neoplasias Ovarianas/complicações , Puberdade Precoce/etiologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Criança , Feminino , Humanos , Neoplasias Ovarianas/patologia , Tumores do Estroma Gonadal e dos Cordões Sexuais/patologiaRESUMO
OBJECTIVE: to evaluate the different presentations of hyperandrogenism produced by ovarian tumors in women at different life stages DESIGN: case report. SETTING: academic institutions. PATIENT(S): 3 patients at different life stages, with increased androgen levels. INTERVENTION(S): review of hospital records. MAIN OUTCOME MEASURE(S): clinical and biochemical features, treatment and follow-up. RESULT(S): a 10 year-old girl with Leydig cell tumor presented with hyperandrogenemia, virilization and changes in social behavior. Another patient, at reproductive age, with a tumor>10 cm, presented with signs of virilization and abdominal mass, whose pathologic analysis disclosed a carcinoid tumor of the ovary associated with stromal hyperplasia. The third patient was a postmenopausal woman with severe alopecia, who presented a steroid cell tumor, rare at that age. CONCLUSION(S): the evaluation of women with signs and symptoms of virilization should include a detailed clinical record, thorough physical examination and an appropriate hormonal dosage, especially when images are inconclusive.
Objetivo: evaluar las diferentes manifestaciones del hiperandrogenismo tumoral de origen ovárico en distintos grupos etarios. Diseño: reporte de casos.Lugar de trabajo: centros académicos.Pacientes: son reportadas tres pacientes con exceso de andrógenos. Resultados: describimos una paciente de 10 años con hiperandrogenemia y signos de masculinización secundarios a un tumor de células de Leydig; otra paciente, en edad fértil, con un tumor carcinoide de ovario asociado a hiperplasia estromal que se manifestó con signos de masa abdominal y de virilización. El tercer caso una mujer, en etapa postmenopáusica con alopecia severa, tenía un tumor de células esteroideas, raro en este grupo etario. onclusión: la evaluación de una mujer con signos y síntomas de virilización debe incluir una detallada historia clínica, examen físico y apropiados dosajes hormonales, especialmente si existe dificultad en establecer su origen cuando los estudios imagenológicos son normales.
Assuntos
Hiperandrogenismo/etiologia , Neoplasias Ovarianas/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Adulto , Criança , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
OBJECTIVE: to evaluate the different presentations of hyperandrogenism produced by ovarian tumors in women at different life stages DESIGN: case report. SETTING: academic institutions. PATIENT(S): 3 patients at different life stages, with increased androgen levels. INTERVENTION(S): review of hospital records. MAIN OUTCOME MEASURE(S): clinical and biochemical features, treatment and follow-up. RESULT(S): a 10 year-old girl with Leydig cell tumor presented with hyperandrogenemia, virilization and changes in social behavior. Another patient, at reproductive age, with a tumor>10 cm, presented with signs of virilization and abdominal mass, whose pathologic analysis disclosed a carcinoid tumor of the ovary associated with stromal hyperplasia. The third patient was a postmenopausal woman with severe alopecia, who presented a steroid cell tumor, rare at that age. CONCLUSION(S): the evaluation of women with signs and symptoms of virilization should include a detailed clinical record, thorough physical examination and an appropriate hormonal dosage, especially when images are inconclusive.
Assuntos
Adulto , Criança , Feminino , Humanos , Pessoa de Meia-Idade , Neoplasias Ovarianas/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Hiperandrogenismo/etiologiaRESUMO
The sclerosing stromal tumor of the ovary is an extremely rare benign tumor more common in young women and without specific symptoms in most cases. Less than 150 cases have been described, of which 8 were diagnosed during pregnancy. In this report, we describe the association between sclerosing stromal tumor of the ovary, Meigs' syndrome and elevated levels of CA-125 in term pregnancy.
Assuntos
Síndrome de Meigs/complicações , Síndrome de Meigs/diagnóstico , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Complicações na Gravidez/diagnóstico , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/diagnóstico , Adolescente , Antígeno Ca-125/sangue , Feminino , Humanos , Síndrome de Meigs/sangue , Neoplasias Ovarianas/sangue , Gravidez , Complicações na Gravidez/sangue , Tumores do Estroma Gonadal e dos Cordões Sexuais/sangueRESUMO
O tumor estromal esclerosante de ovário é uma neoplasia benigna extremamente rara, mais frequente em mulheres jovens e sem sintomas específicos na maioria dos casos. Menos de 150 casos foram descritos, dos quais 8 diagnosticados durante a gestação. Neste relato, documentamos a associação entre tumor estromal esclerosante de ovário, síndrome de Meigs e elevação dos níveis de CA-125 em gravidez a termo.
The sclerosing stromal tumor of the ovary is an extremely rare benign tumor more common in young women and without specific symptoms in most cases. Less than 150 cases have been described, of which 8 were diagnosed during pregnancy. In this report, we describe the association between sclerosing stromal tumor of the ovary, Meigs' syndrome and elevated levels of CA-125 in term pregnancy.
Assuntos
Adolescente , Feminino , Humanos , Gravidez , Síndrome de Meigs/complicações , Síndrome de Meigs/diagnóstico , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/diagnóstico , Complicações na Gravidez/diagnóstico , Tumores do Estroma Gonadal e dos Cordões Sexuais/complicações , Tumores do Estroma Gonadal e dos Cordões Sexuais/diagnóstico , /sangue , Síndrome de Meigs/sangue , Neoplasias Ovarianas/sangue , Complicações na Gravidez/sangue , Tumores do Estroma Gonadal e dos Cordões Sexuais/sangueRESUMO
Conocido antiguamente como el "asesino silencioso", el cáncer de ovario muestra un espectro de síntomas inespecíficos que, una vez reconocidos, facilitan un diagnóstico temprano y prolongan la supervivencia de la paciente. Se presenta el caso de una paciente de 57 años referida de consulta privada por sangrado post menopaúsico con US transvaginal que revela datos de malignidad ovárica, laboratorios de rutina y marcadores tumorales normales...