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1/9. 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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2/9. 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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3/9. 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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4/9. dysgerminoma of the ovary with rhabdomyosarcoma. Report of a case.

    A 14-year-old girl with a large left ovarian mass underwent excision of the tumor which on histologic examination revealed dysgerminoma mixed with elements of rhabdomyosarcoma. A subsequent staging laparotomy revealed a solitary 1-cm nodule along the pelvic wall. This was composed of rhabdomyosarcoma. review of literature revealed that soft tissue sarcomas of several types may on occasion be seen in association with gonadal and extragonadal mixed germ cell tumors or in spermatocytic seminoma of the testis. However, no previously published report of a sarcoma arising in a pure dysgerminoma was found in the literature.
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keywords = dysgerminoma
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5/9. Ovarian dysgerminoma with embryonal carcinoma: A case report.

    The association of an ovarian dysgerminoma with embryonal carcinoma in a 19-year-old woman is described. This is a highly malignant combination resulting in very rapid tumour growth and dissemination. A case report is presented and the aetiology, malignant potential and subsequent management of this most unusual tumour are discussed.
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keywords = dysgerminoma
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6/9. ataxia-telangiectasia with dysgerminoma of right ovary, papillary carcinoma of thyroid, and adenocarcinoma of pancreas.

    The first case of ataxia-telangiectasia (AT) with dysgerminoma of the right ovary, papillary carcinoma of the thyroid, and adenocarcinoma of the pancreas is reported. There is the characteristic trend of the occurrence of the malignant neoplasms in AT. Lymphoreticular neoplasms and leukemia are most frequently seen in the younger patients with AT. On the other hand, epithelial malignant neoplasms are frequently seen in the longer survivors with AT. literature also shows that nucleomegaly is due to precocious or abnormal aging.
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keywords = dysgerminoma
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7/9. gonadoblastoma with dysgerminoma in one ovary and gonadoblastoma with dysgerminoma and yolk sac tumor in the contralateral ovary in a girl with 46XX karyotype.

    A case of gonadoblastoma with dysgerminoma in the left ovary and gonadoblastoma with dysgerminoma and yolk sac tumor in the right of a 10-year-old girl is reported. Her karyotype was 46XX, normal female type. A case of a gonadoblastoma without a y chromosome is very rare. Furthermore, the prognosis for gonadoblastoma accompanied by germ cell tumors other than dysgerminoma was very poor in the past, but the patient remains well 25 months after the first operation.
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keywords = dysgerminoma
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8/9. 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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9/9. The association of ovarian dysgerminoma and gonadoblastoma in a phenotypic female with 46 XY karyotype.

    dysgerminoma is the most common malignant germ cell tumor of the ovary. It can be found either in a pure form or mixed with other germinal elements. It has also been reported arising from gonadoblastoma, which may be regarded as an in situ germ cell malignancy. We describe the case of an 18-year-old patient who had a dysgerminoma arising from the left ovary and a gonadoblastoma replacing the right ovary. She was a nonvirilized phenotypic female, with 46, XY genotype. The patient underwent bilateral salpingo-oophorectomy with total hysterectomy, appendicectomy, infracolic omententectomy, selective pelvic and para-aortic lymphadenectomy, peritoneal random biopsies. The surgical-pathological stage of dysgerminoma was retroperitoneal stage III. The patient received postoperative radiotherapy on the whole pelvis and para-aortic lymph nodes and precautional radio therapy on the mediastinum and supraclavicular lymph nodes. She is currently alive with no evidence of disease after 46 months from surgery.
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