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1/63. Testicular fibroma of gonadal stromal origin with minor sex cord elements: clinicopathologic and immunohistochemical study of 2 cases.

    OBJECTIVE: To report the histologic and immunohistochemical features of 2 cases of intratesticular fibromatous tumors. RESULTS: Microscopically, these tumors were composed of short, randomly interweaving fascicles of spindle cells dispersed within a fibrocollagenous stroma. A sex cord component was detected in one case by microscopic examination and in both cases by immunohistochemical study using MIC2 and anti-inhibin antibodies. CONCLUSIONS: The presence of minor sex cord elements, morphologically or by immunohistochemistry, suggests that these fibromatous tumors are related to and are a subset of sex cord-stromal tumors. Intratesticular fibromatous tumors, of which 11 other cases lacking sex cord elements have been reported, could be considered as the testicular equivalent of ovarian fibroma. These tumors could then be referred to as testicular fibroma of gonadal stromal origin, with or without minor sex cord component.
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2/63. Sonographic presentation of uterine sex cord-stromal tumor.

    A 24-year-old woman presented with continuous vaginal bleeding and anemia. Pelvic sonography demonstrated an enlarged uterus measuring 8.9 x 4.9 x 4.7 cm and a mass within the endometrial cavity measuring 3.0 x 1.8 cm. The mass appeared to be polypoid and echogenic, contained a small cyst, and was suggestive of a polyp. The patient underwent total vaginal hysterectomy. Pathologic examination revealed a uterine sex cord-stromal tumor. To our knowledge, the sonographic features of uterine sex cord-stromal tumor have not been previously reported. The sonographic appearance mimicked that of a polyp.
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3/63. Primary pure intratesticular fibrosarcoma.

    Testicular sarcoma appears to be a rare stromal tumor usually of indolent course with potential for distant metastases. A pure primary intratesticular fibrosarcoma in a 71-year-old male is presented. By the time the tumor became evident metastases had already occurred widely. The course was rapidly fatal. A review of the literature yielded only one case reported previously. The present case illustrates that pure fibrosarcoma occurs occasionally as a stromal tumor and may be associated with a dismal prognosis. The diagnosis of pure fibrosarcoma should be made only after extensive sampling of the testicular tumor to rule out an associated germ cell component. The differential diagnosis also includes the fibroma of gonadal stroma origin and the unclassified sex cord-stromal tumor with a predominance of spindle cells.
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4/63. Loss of the Y-chromosome in the primary metastasis of a male sex cord stromal tumor: pathogenetic implications.

    The first published chromosomal pattern of the retroperitoneal lymph node metastasis of a malignant gonadal stroma cell tumor of the adult testis is presented. karyotyping showed structural chromosomal abnormalities and loss of the Y-chromosome. This loss was confirmed in primary tumor and metastasis using fluorescence in situ hybridization (FISH). The characteristic chromosomal abnormality of adult testicular germ cell tumors, an i(12p), was not present. The results are compared with other data of testicular and ovarian sex cord stromal tumors. From the comparison of the male tumors, it is concluded that loss of the Y-chromosome might have a pathogenetic significance.
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5/63. Ovarian steroid cell tumors, not otherwise specified: a case report and literature review.

    Steroid cell tumors, not otherwise specified, are rare ovarian sex cord-stromal tumors with malignant potential. The majority of these tumors produce steroids with testosterone being the most common. A case of a 46-year-old woman who presented with sudden onset of virilization and a pelvic mass is reported. Various aspects of the presentation, diagnosis, and treatment of these tumors are discussed.
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6/63. Androgen-producing ovarian tumors: a clinicopathological study of 3 cases.

    The clinical course and pathological findings of 3 rare cases of androgen-producing ovarian tumors are presented. The ages of the 3 patients (Cases 1, 2, and 3, respectively) were 43, 34, and 57 years, respectively. Their preoperative serum testosterone levels were 506, 491, and 231 ng/dl, respectively. The pathological diagnoses of Cases 1, 2, and 3 were a Sertoli-stromal cell tumor of intermediate differentiation, a stromal tumor containing leydig cells, and a stromal tumor with minor sex cord elements, respectively. Patient 1 experienced a recurrence, of a lesion at the vaginal stump 1 year and 2 months after the initial surgery. The clinical courses of Cases 2 and 3 have been non-contributory.
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7/63. Malignant ovarian sex cord tumor with annular tubules in a patient with peutz-jeghers syndrome: a case report.

    The majority of ovarian sex cord tumors with annular tubules (SCTAT) are benign neoplasms that arise sporadically. In patients who have peutz-jeghers syndrome (PJS), ovarian SCTAT is often an incidental finding. Malignant behavior in SCTAT has heretofore been reported only in sporadic cases. We report a case of bilateral, malignant SCTAT developing in a 47-year-old woman who had PJS, originally diagnosed as adenocarcinoma on cervicovaginal cytology. Cervicovaginal and peritoneal fluid cytologic preparations were characterized by pseudopapillary clusters and three-dimensional tubes of tumor cells with scanty cytoplasm and high nuclear: cytoplasmic ratio. Examination of surgical resection specimens revealed bilateral, solid ovarian tumors composed of simple and complex annular tubules with hyaline cores, typical of SCTAT. Tumor emboli were present within salpingeal lymphovascular spaces and in both right and left pelvic lymph nodes. flow cytometry of tumor cells demonstrated a diploid phenotype. This case represents the first documented example of bilateral, malignant SCTAT arising in a patient who had PJS, presenting with an atypical cervicovaginal smear.
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8/63. Androgen-secreting steroid cell tumor of the ovary.

    We present the case of a 93-year-old virilized woman with an androgen-secreting ovarian tumor. This rare ovarian sex cord stromal tumor behaved in a malignant fashion. Various aspects of the presentation, diagnosis, and treatment of these tumors are discussed.
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9/63. MRI findings of a case of uterine tumor resembling ovarian sex-cord tumors coexisting with endometrial adenoacanthoma.

    A case of uterine tumor resembling ovarian sex-cord tumors is reported. MRI was performed for the staging of the endometrial adenoacanthoma diagnosed by biopsy. A well-defined, slightly hyperintense mass with central hypointensity was noted in the myometrium on T2-weighted images. Pathological diagnosis of the myometrial mass was uterine tumor resembling ovarian sex-cord tumors. The central hypointense area was a leiomyomatous component. Adenoacanthoma was also confirmed. These features of coexisting tumors correlated well with the MRI findings.
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10/63. Uterine tumor resembling ovarian sex-cord tumor--a case report and review of the literature.

    A uterine tumor resembling an ovarian sex-cord tumor (UTROSCT) is a very rare lesion with only 38 cases reported in the literature so far. Here, we show an additional case of a pure UTROSCT with a dna stemline at 1c in a 49-year-old woman presenting with abnormal vaginal bleeding. Problems in differential diagnosis arise mainly due to the variable histological picture of UTROSCT. Immunohistochemically, these tumors express cytokeratin, epithelial membrane antigen, vimentin, and smooth muscle actin. Moreover, in some cases, CD99 and alpha-inhibin are detectable. Although 36% of UTROSCT have infiltrative margins, almost all of them behave benignly. It is thus questionable whether the same prognostic criteria apply for these tumors as for endometrial stromal sarcomas. However, in the so-called mixed UTROSCT, the endometrial stromal sarcoma component determines the outcome.
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