RESUMO
Conceitualmente, as gônadas disgenéticas são gônadas que não sofreram uma completa diferenciação. Em vista disso, constituem parte de uma ampla gama de entidades clínicas possuidoras de fenótipos e de genótipos diversos. Seus cariótipos contêm o cromossomo Y ou seus fragmentos, ou raramente não os contêm. Essas alterações geram maior risco para a ocorrência de neoplasias nessas gônadas. Na sequência deste estudo apresentamos as neoplasias mais comumente associadas aos diversos tipos de disgenesias gonadais. A neoplasia mais comum é o gonadoblastoma e outros como os disgerminomas e os tumores do seio endodérmico também podem estar associados. A detecção dessas anormalidades de modo precoce é o que nos motivou para a presente revisão
By definition, dysgenetic gonads are those that did not undergo a complete differentiation. They make up a vast array of clinical entities, having different phenotypes and genotypes. Their kariotypes contain the Y chromosome or fragments of it, and, in rare cases, do not contain it. Such alterations generate greater potential for the occurrence of neoplasms in such gonads. This study presents neoplasms which are most commonly associated with several types of gonadal dysgenesis. The most common neoplasia is gonadoblastoma and others like disgerminoma or yolk sac tumors may be associated. The early detection of such potential is the reason for this review
Assuntos
Humanos , Masculino , Feminino , Células Germinativas/patologia , Disgenesia Gonadal/complicações , Disgerminoma/etiologia , Gonadoblastoma/etiologia , Tumor do Seio Endodérmico/etiologia , Disgenesia Gonadal Mista , Gônadas/anormalidades , Síndrome de TurnerRESUMO
Gonadoblastoma (GB) is an in situ tumor consisting of a heterogeneous population of mature and immature germ cells, other cells resembling immature Sertoli/granulosa cells, and Leydig/lutein-like cells, may also be present. GB almost exclusively affects a subset of patients with intersex disorders and in 30% of them overgrowth of the germinal component of the tumor is observed and the lesion is term dysgerminoma/seminoma. Several pathways have been proposed to explain the malignant process, and abnormal OCT3/4 expression is the most robust risk factor for malignant transformation. Some authors have suggested that OCT3/4 and beta-catenin might both be involved in the same oncogenic pathway, as both genes are master regulators of cell differentiation and, overexpression of either gene may result in cancer development. The mechanism by which beta-catenin participates in GB transformation is not completely clear and exploration of the E-cadherin pathway did not conclusively show that this pathway participated in the molecular pathogenesis of GB. Here we analyze seven patients with mixed gonadal dysgenesis and GB, in an effort to elucidate the participation of beta-catenin and E-cadherin, as well as OCT3/4, in the oncogenic pathways involved in the transformation of GB into seminoma/dysgerminoma. We conclude that the proliferation of immature germ cells in GB may be due to an interaction between OCT3/4 and accumulated beta-catenin in the nuclei of the immature germ cells.
Assuntos
Caderinas/fisiologia , Disgerminoma/etiologia , Disgenesia Gonadal Mista/complicações , Gonadoblastoma/etiologia , Fator 3 de Transcrição de Octâmero/fisiologia , Neoplasias Ovarianas/etiologia , Neoplasias Testiculares/complicações , beta Catenina/fisiologia , Adolescente , Transformação Celular Neoplásica , Pré-Escolar , Feminino , Humanos , Imuno-Histoquímica , Lactente , MasculinoRESUMO
Siendo el Disgerminoma el tumor de células germinales más común en mujeres jóvenes, es también de los menos frecuentes de los tumores de ovario, oscilando su incidencia en Colombia del 0.48 a 0.55 por ciento. Se presentan dos casos atendidos en el Departamento de Ginecología y Obstetricia del Hospital Universitario Metropolitano en un período de 10 años
Assuntos
Humanos , Feminino , Adulto , Disgerminoma/complicações , Disgerminoma/diagnóstico , Disgerminoma/epidemiologia , Disgerminoma/etiologia , Disgerminoma/patologia , Disgerminoma/fisiopatologia , Disgerminoma/cirurgiaRESUMO
Malignant tumours of the testis are reported to be rare in negroes. All cases reported in the records of the University Hospital of the West Indies and the Jamaica Cancer Registry over the period June 1958 to December 1976 were studied. There were 26 cases, giving a world standardised incidence of 0.40 per 100,000 per year. This incidence is higher than that seen in African negroes but lower than that of negroes in North America. The peak age incidence is a decade later than that commonly seen in high incidence countries.
Assuntos
Disgerminoma/epidemiologia , Teratoma/epidemiologia , Neoplasias Testiculares/epidemiologia , Adolescente , Adulto , África , Fatores Etários , População Negra , Disgerminoma/etiologia , Humanos , Jamaica , Masculino , Pessoa de Meia-Idade , América do Norte , Sistema de Registros , Temperatura , Teratoma/etiologia , Neoplasias Testiculares/etiologia , População BrancaRESUMO
Malignant tumours of the testis are reported to be rare in negroes. All cases reported in the records of the University Hospital of the West Indies and the Jamaica Cancer Registry over the period June 1958 to December 1976 were studied. There were 26 cases, giving a world standardised incidence of 0.40 per 100,000 per year. This incidence is higher than that seen in African negroes but lower than that of negroes in North America. The peak age incidence is a decade later that commonly seen in high incidence countries (AU).