Cases reported "Turner Syndrome"

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1/8. dysgerminoma in Turner's syndrome.

    The importance of the Y-chromosome for the germ cell tumour development in gonadal dysgenesis has been emphasized many times. In contrast, only two cases of dysgerminoma or gonadoblastoma had been published so far in the XO-Turner's syndrome. With this report, another case of Turner's syndrome developing a dysgerminoma in a gonadal streak is presented. No Y-chromosome containing stemline could be detected in the patient nor in the tumour. A primary genetic etiology or a mechanism related to early secondary regression or dysgenesis of the gonad are discussed as causative factors in germ cell tumour development within gonadal streaks.
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2/8. Dysgerminomas (seminomas) in genetic males with female phenotype. One case of gonadal dysgenesis and gonadoblastoma and one of testicular feminization.

    Gonadal tumors in patients with somatosexual ambiguity are rare but of considerable clinical and pathological interest. Two patients with dysgerminomas and abnormal sexual differentiation were recently treated at the Department of Gynecologic Oncology. One (Case 1) was an 18-year old girl and the other (Case 2) an unmarried woman of 58 years. In spite of their female phenotype both showed a male genotype, 46, XY. In Case 1 the dysgerminoma (or rather seminoma) originated in a gonadoblastoma in one of two dysgenetic testes but in Case 2 in one of two "feminizing testes". The clinico-pathological features of both cases will be reported and commented and the relation between the germ cell tumors, gonadal dysgenesis and the Y-chromosome will be discussed.
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3/8. dysgerminoma in 45,X turner syndrome: report of a case.

    Here we report the fourth case of dysgerminoma in a patient with the syndrome of gonadal dysgenesis and 45,X karyotype. Typical Turner's syndrome features were unusually associated with breast development, menarche and secondary amenorrhoea. Exaggerated basal and GnRH stimulated gonadotrophin and low oestradiol levels were typical of post-pubertal Turner's syndrome. Detailed (standard) chromosome and banding analysis excluded the presence of y chromosome material. This case suggests that the presence of a y chromosome is not necessary for abnormal differentiation of germ cells and the occurrence of a gonadoblastoma.
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4/8. h-y antigen in 46,XY pure testicular dysgenesis.

    Clinical, cytogenetic, pathologic, and histocompatibility-Y (H-Y) antigen studies were performed on a phenotype female with primary amenorrhea and streak gonads. Pathological examination of tissues removed at total hysterectomy and bilateral salpinog-gonadectomy showed gonadoblastoma and dysgerminoma of left streak. A single F-body (y chromosome) was found in buccal smears. Analysis of blood cells and tumor fibroblasts showed a 46,XY chromosome constitution (Q-banding). The data were consistent with a diagnosis of 46,XY pure testicular dysgenesis. Positive results for h-y antigen were found in this case.
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5/8. Invasive dysgerminoma in a girl with 45,X/46,X; mar mosaicism.

    We report a 16-year-old girl with features of Turner's syndrome from whom an invasive dysgerminoma was removed. Cytotoxic drugs were given for the next 12 months. mosaicism of two karyotypes (45,X/46,X; mar) was found in various tissues. The literature is reviewed with special regard to cytogenetic findings and prognosis of malignant growth and differentiation of dysgenetic gonads.
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ranking = 2.5
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6/8. dysgerminoma in a pure 45,X turner syndrome: report of a case and review of the literature.

    There is a high risk of neoplasm in dysgenetic gonads. Classically, in turner syndrome, only patients with 45, X/46, XY mosaicism karyotype or with a fragment of y chromosome (45, X mar) are at risk of developing gonadal tumor. A case of a dysgerminoma arisen on the dysgenetic gonad of a 45, X Turner patient in the absence of y chromosome material at cytogenetic and molecular biology analysis is reported. Five cases of gonadal tumor with a pure 45, x chromosome constitution have been previously reported in the literature. In these cases only cytogenetic analysis was performed. This is the first case of an ovarian tumor in a 45, X turner syndrome in which the presence of Y material can be ruled out by an extensive molecular analysis of the blood and the tumor.
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7/8. Unique combination of an ovarian gonadoblastoma, dysgerminoma, and mucinous cystadenoma in a patient with Turner's syndrome: a cytogenetic and molecular analysis.

    Phenotypically female patients with a (mosaic) XY karyotype are at high risk to develop gonadoblastoma with potential progression to dysgerminoma. We studied a Turner's syndrome patient with a composite ovarian neoplasm of a gonadoblastoma, a dysgerminoma, and a mucinous cystadenoma. Nonradioactive in situ hybridization showed that the patient had a XO/XY genotype with deletion of part of Yq. Molecular analysis located the chromosomal breakpoint in deletion interval 6, indicating that potential genes responsible for the development of gonadoblastoma may be located on the short arm of the y chromosome or on the long arm, centromeric of deletion interval 6. Moreover, using the XO/XY mosaicism as a clonal marker, the dysgerminoma and the mucinous cystadenoma were shown to be of independent origin. Therefore, in this case, we find support for the hypothesis that mucinous cysts with gastrointestinal epithelium can be of ovarian surface epithelial cell origin. This case also demonstrated that the occurrence of a composite tumor does not unequivocally imply that both components are of the same origin. Clonal analysis is required to determine the relation of the tumor constituents.
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keywords = dysgerminoma
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8/8. Familial occurrence of gonadal tumors in XY females with breast development.

    Three sisters with XY gonadal dysgenesis are presented. All of them have primary amenorrhea and breast development, and in two, a gonadal tumor, seminoma (dysgerminoma) was found. The danger of gonadal neoplasm in XY females with normal female build is emphasized.
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