Cases reported "Dysgerminoma"

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11/154. Isolated duodeno-pancreatic involvement due to metastatic dysgerminoma ovary and its management by a modified pancreatico-duodenal resection.

    Dysgerminomas of the ovary rarely metastasize to abdominal viscera and when they do, the involvement is a part of a disseminated disease. A 30-year-old woman developed isolated duodenopancreatic dysgerminoma 14 years after salpingo-oophorectomy. The clinical picture was complicated by the presence of tuberculous lesions in the liver which mimicked metastatic disease. Surgical excision was carried out using a modified pancreatic head resection.
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12/154. 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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13/154. microsatellite instability in gonadal tumors of XY pure gonadal dysgenesis patients.

    To investigate genetic alternation accompanied by malignant transformation in gonadal tumors of XY pure gonadal dysgenesis patients, we investigated microsatellite instability in the hMSH1, hMSH2, TP53, and DCC loci, and ras mutations in two patients. The gonadal tumors from the patients were combined gonadoblastoma and dysgerminoma. microsatellite instability and/or loss of heterozygotes (LOH) at hMSH1, hMSH2, and TP53 were detected in the dysgerminoma lesions of the both patients, but were not observed in any normal tissues. In the analyses of the H-, K-, or N-ras genes, where specific mutations have been frequently reported, no mutations were observed in the tumors. It is suggested therefore that microsatellite instability plays an important role in malignant transformation of gonadal tumors in patients with XY pure gonadal dysgenesis.
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14/154. gonadoblastoma and dysgerminoma associated with XY gonadal dysgenesis in an adolescent with chronic renal failure: a case of frasier syndrome.

    STUDY OBJECTIVES: To report a rare reason for primary amenorrhea, a frasier syndrome, XY gonadal dysgenesis associated with renal failure with eventual development of gonadoblastoma. To study immunohistochemical analysis of gonadoblastoma and dysgerminoma. To analyze the possibility of androgen receptor mutation in this rare syndrome. methods: We report a case of a 16-yr-old female with this syndrome. She underwent a laparoscopic bilateral gonadectomy and salpingectomy. A histopathological examination revealed gonadoblastoma with focal malignant dysgerminoma in the left dysgenetic gonad and an immunohistochemical of these fairly rare, malignant tumors. An androgen receptor was coded. Analysis was done. RESULTS: Immunohistochemical analysis showed that inhibin was strongly positive in gonadoblastoma but negative in dysgerminoma. No mutations of the androgen receptor gene were found. CONCLUSIONS: Inhibin positivity in gonadal stroma and in gonadoblastoma may indicate hormonal activity causing advanced puberty in patients with XY gonadal dysgenesis.
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15/154. XY female with a dysgerminoma and no mutation in the coding sequence of the SRY gene.

    We report a 46,XY 11-year-old girl with pure gonadal dysgenesis who developed a dysgerminoma. The testis-determining gene SRY, a candidate for sex reversal, whose alterations seem to correlate with dysgerminoma, was analyzed and found to be normal; its coding sequence was negative for deletions and mutations. DMRT-1 gene mapping on 9p and DAX-1 on Xp21 were also normal. These results suggest the involvement of other genes in sex reversal and call into question the putative relationship between SRY alterations and dysgerminoma.
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16/154. dysgerminoma in a patient with a tumor of the neck. Empiric treatment of stage IV dysgerminoma.

    The evolution of therapy for malignant ovarian germ cell tumors is one of the true success stories in oncology. treatment outcome has improved greatly thanks to cisplatin-based combination chemotherapy. According to the well-established treatment guidelines for advanced cases, we treated a case of stage IV undifferentiated germ cell tumor in which we were able to preserve the patient's fertility. We concluded that the PEP regimen is an effective treatment for the patient with metastatic germ cell tumor.
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17/154. dysgerminoma with a slightly elevated alpha-fetoprotein level diagnosed as a mixed germ cell tumor after recurrence.

    A pure dysgerminoma shows a normal serum alpha-fetoprotein level, and mixed germ cell tumors containing endodermal sinus tumor elements have elevated serum alpha-fetoprotein levels, ranging from >100 to far higher than 1,000 ng/ml. A 40-year-old woman was diagnosed as having a stage Ia pure dysgerminoma with a slight alpha-fetoprotein elevation (11 ng/ml), after a staging laparotomy, because we could not find any yolk sac element in the original tumor. After 44 months, she had a pelvic recurrent tumor with a significant elevation of the serum alpha-fetoprotein concentration (1,520 ng/ml); histological examination of a needle biopsy specimen revealed a typical yolk sac tumor. Eventually, her initial tumor was diagnosed as a mixed germ cell tumor. The patient was successfully treated with seven courses of chemotherapy and has been disease free for 22 months. It is necessary to be aware of the possibility of a mixed germ cell tumor containing a yolk sac element, even when the alpha-fetoprotein level is only slightly elevated.
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18/154. Peripheral neuropathy in association with an ovarian dysgerminoma.

    BACKGROUND: Peripheral neuropathy among women with ovarian dysgerminoma has not been so far reported in the literature. CASE: We report a case of an 18-year-old woman with an association of an abdominal relapse of an ovarian dysgerminoma and a polyradiculoneuritis. The normality of neurologic investigations and the disappearance of all sensitive and muscular alterations after tumor ablation and one cycle of chemotherapy are in favor of a paraneoplastic syndrome. After 5 years this young woman is still in complete remission without any neurologic symptoms. CONCLUSION: We describe the first case of a polyradiculoneuritis associated with an ovarian dysgerminoma. The evolution of the paraneoplastic syndrome was favorable after treatment of the tumor.
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19/154. 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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20/154. A case of mixed-type dysgerminoma with a high serum concentration of both human chorionic gonadotropin and alpha-fetoprotein in a child.

    dysgerminoma is divided into two types: pure and mixed. The mixed type is related to other various elements of germ cell tumors. We experienced a case of mixed type dysgerminoma with a high serum concentration of both human chorionic gonadotropin and alpha-fetoprotein. The patient was a 6 year old girl who was admitted to the Hamamatsu University School of medicine with an abdominal mass. Laboratory investigations revealed elevated serum alpha-fetoprotein and high concentration of serum beta-human chorionic gonadotropin. The tumor originated from the left ovary. The histopathological diagnosis was dysgerminoma. serum human chorionic gonadotropin and alpha-fetoprotein levels were useful markers in monitoring the response to treatment in this patient.
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