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1/6. Steroid cell tumour not otherwise specified during pregnancy: a case report and diagnostic work-up for virilisation in a pregnant patient.

    Steroid cell tumours not otherwise specified are rare ovarian tumours, which can cause foetal and maternal virilisation. This is the first case report that describes a steroid cell tumour not otherwise specified during pregnancy. Differential diagnosis, a diagnostic work-up and treatment are discussed.
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keywords = cell tumour
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2/6. Testicular sex cord-stromal tumours: the Edinburgh experience 1988-2002, and a review of the literature.

    AIMS: sex cord-stromal tumours of the testis are uncommon tumours, accounting for around 5% of testicular neoplasms. Treatment is primarily surgical, with no adjuvant therapy of proven benefit. We present a single-centre experience over a period of 15 years. MATERIALS AND methods: From 1988 to 2002, 18 patients with a diagnosis of sex cord-stromal tumour were referred to our centre. A retrospective analysis of their case notes was made and a pathological review undertaken. RESULTS: Sixteen were Leydig-cell tumours and two were Sertoli cell. For the Leydig-cell tumours, the median age at presentation was 42 years, 50% presented with a testicular mass and 31% with gynaecomastia. Two patients followed a malignant course: one revealing disease dissemination at initial staging, and a second 12 months after potentially curative orchidectomy. Salvage retroperitoneal lymphadenectomy in the latter patient proved unsuccessful. Clinical outcome correlated strongly with the presence of adverse pathological features described previously in the literature. After a median follow-up of 46 months, two patients have developed progressive disease, and two patients have died, one of metastatic Leydig-cell tumour. No patient defined as being of low malignant potential on pathological examination has relapsed outside our review period of 2 years. CONCLUSION: We confirm the overall excellent prognosis for most of the patients with sex cord-stromal tumours of the testis. Compared with most previous reports, pathological features seem to predict with reasonable accuracy the risk of malignant behaviour, and can adequately inform the subsequent review policy.
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keywords = cell tumour
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3/6. Uterine retiform sertoli-leydig cell tumor: report of a case providing additional evidence that uterine tumors resembling ovarian sex cord tumors have a histologic and immunohistochemical phenotype of genuine sex cord tumors.

    We report a case of a retiform sertoli-leydig cell tumor of intermediate differentiation presenting as a uterine intracavity polypoid mass in a 63-year-old woman. In contrast to sertoliform endometrioid carcinoma and to hitherto reported uterine tumors resembling ovarian sex cord tumors (UTROSCTs), which are primarily characterized by tubular glands and solid tubules, this tumor, which most likely represents a UTROSCT, showed a large spectrum of histologic features typical of a genuine retiform sertoli-leydig cell tumor. The diagnosis was confirmed by a battery of immunohistochemical stains, which also served as a tool for differential diagnosis with other neoplasms. The tumor cells were positive for broad spectrum keratin (CK) CK18, vimentin, calretinin, and progesterone receptor. Only a few isolated cells stained for inhibin. The tumor cells were negative for CK7, CK5/6, epithelial membrane antigen (EMA), carcinoembryonic antigen (CEA), carbohydrate antigen 125 (CA125), thrombomodulin, 013 (CD99), melan A, alpha-fetoprotein (AFP), placental alkaline phosphatase (PLAP), alpha-1-antitrypsin, estrogen receptor, S100, neurone specific enolase (NSE), chromogranin, synaptophysin, desmin, caldesmon, and CD10. Divergent differentiation of uterine cells seems to be the most likely pathogenetic mechanism. To the best of our knowledge, no UTROSCT showing such a variety of histologic features indicative of a true sex cord tumor has been reported before.
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ranking = 9.4192531777284
keywords = sertoli
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4/6. Testicular tumour with features of sex cord tumor with annular tubules associated with cryptorchidism and infertility--a case report.

    sex cord tumor with annular tubules (SCTAT) is a rare tumor seen in the ovary usually associated with peutz-jeghers syndrome. Testicular SCTAT are more infrequent and only four such cases have been reported in the literature. A 28-year-male presented with infertiliy. He had a history of orchiopexy 10 years back for right-sided inguinal testes. A right testicular biopsy done to investigate the cause of infertility revealed testicular atrophy with a focus suspicious of SCTAT. The right-sided orchiectomy revealed two firm nodules of 0.5 and 0.2 cms of SCTAT and sertoli cell nodule (SCN) respectively on microscopy. He did not have any features of Peutz-Jeghers or any other dysgenetic syndrome. He is asymptomatic after follow up of 26 months. This is the fifth case of testicular SCTAT and the first with SCN.
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ranking = 8.141033877239
keywords = sertoli cell, sertoli
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5/6. Intra-abdominal desmoplastic small cell tumour in a patient with peutz-jeghers syndrome.

    A surgical case of intra-abdominal desmoplastic small cell tumour with divergent differentiation (IDS-CT) in a patient with peutz-jeghers syndrome (PJS) is reported. The patient was a 23-year-old woman who underwent surgery for a tumour in the left paraovarian region. Widespread peritoneal metastases were noted. Histopathological examination revealed solid cell nests composed of medium-sized cells demarcated by desmoplastic stroma. Gland-like spaces were found within many cell nests. Tumour cells were positive for cytokeratin and smooth muscle actin, but negative for desmin. Both ovaries contained minute foci of a sex cord tumour with annular tubules. This is the first documentation of the association of IDSCT with PJS. Since patients with PJS have an increased risk of cancer in various organs, the occurrence of IDSCT in the present patient might not be fortuitous.
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keywords = cell tumour
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6/6. gonadoblastoma with proliferation resembling sertoli cell tumor.

    A gonadoblastoma containing a proliferation of the sex cord element resembling a sertoli cell tumor was found in the dysgenetic gonad of a 19-year-old Japanese phenotypic woman whose karyotype was 46 XY. The tumor was a solid mass 9 cm in maximal dimension composed of gonadoblastoma, dysgerminoma, and a proliferative sex cord element resembling a sertoli cell tumor. Transitions between the gonadoblastoma nests and the sertoliform element were observed. These findings suggest that the sex cord element of gonadoblastoma can progress to an independent sex cord tumor.
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ranking = 1.8838506355457
keywords = sertoli
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