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1/17. 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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2/17. Ovarian sex cord tumor with annular tubules in a woman with premature ovarian failure.

    OBJECTIVE: To report a case of premature ovarian failure (POF) associated with an ovarian sex cord tumor with annular tubules. DESIGN: Case report. SETTING: Reproductive endocrinology unit in a tertiary academic center. PATIENT(S): A 20-year-old woman with POF. INTERVENTION(S): biopsy of the rudimentary ovary by laparoscopy. MAIN OUTCOME MEASURE(S): Protocol for POF investigation and histological study of the ovarian sample. RESULT(S): An ovarian sex cord tumor with annular tubules was detected in the rudimentary right ovary. CONCLUSION(S): The rare ovarian sex cord tumor with annular tubules, which may be hormonally active, was detected in a case of POF.
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3/17. Uterine tumor resembling ovarian sex-cord tumor: report of a case mimicking cervical adenocarcinoma.

    Uterine tumors resembling ovarian sex-cord tumors (UTROSCTs) are unusual neoplasms with histologic features that resemble those within ovarian Sertoli and granulosa cell tumors. We report the case of a 24-year-old woman with a UTROSCT presenting as a cervical mass, which on initial evaluation was thought to represent cervical adenocarcinoma. The patient's cervical biopsy specimen contained epithelioid cells arranged in tubules and anastomosing cords, without significant cellular atypia or mitotic activity. Because this morphology elicited a broad differential diagnosis, immunohistochemical studies were performed. The tumor was found to be diffusely positive for cytokeratin cocktail, calretinin, and desmin, focally positive for CK7 and SMA, and negative for EMA, CEA, inhibin, CD10, CK20, chromogranin, and synaptophysin. Ultrastructural examination revealed occasional gland-like lumens with cells joined by desmosomes and a continuous basal lamina. UTROSCTs have features that may cause them to be confused with more common tumors, especially in limited biopsy samples, and should be included in the differential diagnosis when a gland-forming neoplasm with an unusual appearance is identified in a cervical or endometrial biopsy specimen.
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4/17. Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in peutz-jeghers syndrome.

    peutz-jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Although peutz-jeghers syndrome polyps are hamartomas, frequent association of this syndrome with both gastrointestinal and non-gastrointestinal tumours had led to reassessment of the cancer risk in this hereditary disorder. The most common gynaecological tumors in this syndrome are adenoma malignum of the uterine cervix and ovarian sex cord tumor, particularly sex cord tumor with annular tubules. The question of malignant change in a polyp or of the association of gastro intestinal carcinomas still discuss. The authors report a case of peutz-jeghers syndrome in a young patient who developed a colonic adenocarcinoma in a hamartomatous polyp together with an incidentally discovered bilateral malignant sex cord tumours. We discuss its association with certain benign and malignant tumors and the risk of rare complications of these hamartomatous polyps. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the literature.
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5/17. 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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6/17. 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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7/17. Chemotherapy-induced alterations in ovarian sex cord tumor with annular tubules (SCTAT): a diagnostic challenge.

    We present a case of ovarian sex cord tumor with annular tubules (SCTAT) in a patient that received neoadjuvant chemotherapy with 3.5 years of follow-up. Neo-adjuvant chemotherapy is a common practice in advanced cases of ovarian carcinoma and is rarely applied in nonepithelial ovarian tumors. Chemotherapy-induced morphologic alterations of the subsequently resected tissue samples are well recognized; in this case, chemotherapy-induced cytoarchitectural changes created diagnostic difficulties in the subsequently resected specimen.
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8/17. Ovarian sex cord tumour with annular tubules diagnosed by fine needle aspiration cytology--a case report.

    sex cord tumor with annular tubules (SCTAT) is a rare ovarian neoplasm with distinctive morphological features whose cytological features have rarely been described. We report a case of SCTAT diagnosed on fine needle aspiration cytology (FNAC) with a brief review of literature. A 35 year old female presented with recurrent abdominal mass. Fine needle aspiration was done under ultra sound guidance. Cytology smears showed three dimensional tubular structures, metachomatic hyaline masses and uniform neoplastic cells dispersed and in cohesive clusters. Cell block study showed the characteristic simple and complex tubules leading to the diagnosis. The cytological findings of a rare ovarian neoplasm are described, which is only the third such report in English literature. The cytologic features of ovarian SCTAT are as distinctive as its histologicfeatures. Cell block study brings out the distinctive architecture of the tumor and helps in differentiating it from other closely related tumors.
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9/17. endoscopic ultrasound-guided fine needle aspiration diagnosis of metastatic sex cord tumor with annular tubules: a case report.

    The cytologic findings of a paratracheal metastasis from a malignant sex cord tumor with annular tubules (SCTATs) diagnosed by endoscopic ultrasound guided fine needle aspiration are described. Cytologic features of SCTATs include the presence of highly cellular aspirates forming simple and complex rosette-like structures around central rounded hyaline material, small nucleoli, and prominent nuclear grooves. These cytologic findings are clearly distinct from the poorly formed rosette-like structures of granulosa-cell tumors and are reminiscent of the low-power appearance of the cribriform variant of adenoid cystic carcinoma. The fine needle aspiration cytologic features of only two other cases have been previously described and, to the best of our knowledge, this is the first case reporting the cytologic diagnosis of a distant metastasis of a SCTATs by endoscopic ultrasound-guided fine needle aspiration. In this case, the distinctive and characteristic cytologic features have allowed the proper diagnosis of a distant metastasis of SCTATs.
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10/17. peutz-jeghers syndrome with multiple genital tract tumors and breast cancer: a case report with a review of literatures.

    We report here on the multiple genital tract neoplasms in a 41-yr-old Korean woman with peutz-jeghers syndrome (PJS). The patient presented with lower abdominal pain. Her previous medical history was PJS and breast cancer. Pelvic ultrasound showed a multilocular cyst at the right adnexal region, diagnosed as bilateral ovarian mucinous borderline tumors. An ovarian sex cord tumor with annular tubules was incidentally diagnosed together with a minimal deviation adenocarcinoma of the uterine cervix and mucinous metaplasia of both the Fallopian tubal mucosa and the endometrium. Although the cases of multiple genital tract tumors with PJS has rarely been reported, the present case appears to be the first in korea in which the PJS syndrome was complicated by multiple genital tract tumors and infiltrating carcinoma of the breast. The clinical significance of the multiple genital tract tumors and breast cancer associated with PJS is reviewed.
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