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1/18. gonadoblastoma associated with embryonal carcinoma in an anatomically normal man.

    A case of gonadoblastoma associated with embryonal carcinoma in a normally decended testis of an anatomically normal 20-year-old man is reported. The patient had enlargment of the supraclavicular lymph nodes, which contained metastatic yolk sac tumor. Pulmonary and abdominal metatases were present. Despite chemotherapy and radiotherapy the patient died 9 months after presentation. review of the literature concerning gonadoblastoma in phenotypic male patients is presented.
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ranking = 1
keywords = gonadoblastoma
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2/18. ataxia-telangiectasia with ovarian gonadoblastoma and contralateral dysgerminoma.

    Although neoplasms are unusually frequent in patients with ataxia-telangiectasia, the occurrence of primary tumors of the ovary in such patients is exceedingly rare. This report describes a 17-year-old phenotypic female with ataxia-telangiectasia, who was found to harbor an ovarian gonadoblastoma and a contralateral dysgerminoma. The latter tumor has occurred in only one other patient with ataxia-telangiectasia, while an association with gonadoblastoma has never been documented previously. Additional unusual features rarely encountered in patients with gonadoblastoma included origin of the tumor within a histologically proven ovary, and a 46,XX karyotype. The possibility that the dysgerminoma also arose from a gonadoblastoma is discussed.
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ranking = 4
keywords = gonadoblastoma
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3/18. Bilateral seminomas in a 45X/46XY mosaic with Turner's phenotype: an unusual case of mixed gonadal dysgenesis.

    A wide spectrum of phenotypic manifestations are seen in cases with 45X/46XY mosaicism. We present a case with 45X/46XY having female phenotype with Turner's stigmata. Prophylactic laparoscopic gonadectomy was performed and the patient was found to have mixed gonadal dysgenesis with bilateral gonadoblastomas. Microinvasive seminomas were also detected in both gonadoblastomas. The presence of Y cell line in karyotype prompted early and prophylactic gonadectomy, a procedure which is life-saving for these individuals.
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ranking = 1
keywords = gonadoblastoma
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4/18. gonadoblastoma with unusual mixed germ cell overgrowth--a case report.

    gonadoblastoma is an uncommon tumour of ovary occurring exclusively in patients with inter sex disorders. We are presenting an unusual case of gonadoblastoma with distinctly rare pattern of germ cell overgrowth on the other side in an eighteen year old girl.
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ranking = 0.5
keywords = gonadoblastoma
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5/18. gonadoblastoma with contralateral dysgerminoma in a young female--a case report.

    Gonadoblastomas are rare germ cell and sex cord stromal tumours, often associated with dysgerminomas. They occur almost entirely in patients with pure or mixed gonadal dysgenesis and in male pseudohermaphroditism. A 19 year old female was admitted in our hospital for evaluation of primary amenorrhoea. She had poor secondary sexual characters, left sided streak gonad and right sided ovarian tumour. Histopathology showed gonadoblastoma in streak gonad with contralateral dysgerminoma. This case is presented because of its rarity and clinical importance of recognizing such cases because of excellent prognosis.
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ranking = 0.5
keywords = gonadoblastoma
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6/18. Bilateral gonadoblastoma producing steroid hormones in a patient with 45,X/46,XY gonadal dysgenesis.

    A case of bilateral gonadoblastoma in a phenotypic female with 45,X/46,XY gonadal dysgenesis is presented. Hormonal investigations revealed that serum testosterone, estradiol and beta-human chorionic gonadotropin decreased following excision of the tumors, but follicle-stimulating hormone and luteinizing homrone levels increased further. Immunohistochemical staining for testosterone and estradiol was positive in both Leydig and lutein-like cells in the tumor. It is suggested that gonadoblastoma is capable of producing testosterone and estradiol, and Leydig or lutein-like cells may be the actual source of these steroid hormones.
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ranking = 3
keywords = gonadoblastoma
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7/18. True hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, 46,XX/46,XY karyotype, and a successful pregnancy.

    The first case (to the authors' knowledge) is reported of a true hermaphrodite with bilateral ovotestes, bilateral gonadoblastomas and dysgerminomas, a 46, XX/46,XY karyotype, and a successful pregnancy. The true hermaphroditism was diagnosed during infancy. The patient was subsequently found to have a gonadoblastoma and a microscopic dysgerminoma in the gonad diagnosed as an ovotestis and excised during infancy. The successful pregnancy occurred when the patient was 29 years old. A year later a large gonadal tumor affecting the remaining gonad was excised. The gonad was found to be an ovotestis, and the tumor was a dysgerminoma arising from a gonadoblastoma. This case further emphasizes the malignant potential of the y chromosome in patients with abnormal gonads.
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ranking = 3.5
keywords = gonadoblastoma
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8/18. association of microscopic gonadoblastoma and contralateral ovarian fibroadenoma in patients with gonadal dysgenesis and Turner phenotype.

    A rare case of microscopic gonadoblastoma associated with gonadal fibroadenoma in a patient with gonadal dysgenesis and Turner phenotype is reported. The higher incidence of tumor pathologies in patients with gonadal dysgenesis presenting a y chromosome in their karyotype is discussed, and the need for judicious microscopic analysis of the gonadal streaks of these patients for the detection of possible incipient tumors is emphasized.
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ranking = 2.5
keywords = gonadoblastoma
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9/18. 45,X streak gonad syndrome associated with bilateral 'burnt out' gonadoblastoma.

    A 28-year-old woman with well-developed female secondary sexual characteristics, secondary amenorrhea, short stature and a few somatic anomalies of streak gonad syndrome who had bilateral 'burnt out' gonadoblastoma associated with 45,X karyotype is reported. Although the exact source of steroid production in the present case could not be defined, it seems probable that the tumor once secreted steroid hormones which were responsible for the development of the female secondary sexual characteristics. At the time of diagnosis there was no evidence of hormonal activity of the tumor. Cases of gonadoblastoma without y chromosome are reviewed. The diagnostic difficulties encountered in patients with gonadoblastoma not associated with y chromosome are discussed.
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ranking = 3.5
keywords = gonadoblastoma
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10/18. gonadoblastoma associated with malignant teratoma.

    A case of gonadoblastoma associated with malignant teratoma in the contralateral ovary is described. Considerations are offered concerning the malignant potential of dysgenetic gonads as well as the diagnostic and therapeutic approaches in such situations.
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ranking = 0.5
keywords = gonadoblastoma
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