Cases reported "Sertoli Cell Tumor"

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1/18. sertoli cell tumor in androgen insensitivity syndrome--a case report.

    A 26-year-old individual with androgen insensitivity syndrome was operated on for a 3200-g sertoli cell tumor of the left gonad with retroperitoneal metastases. Six courses of bleomycin, etoposide, and cisplatin chemotherapy followed surgical treatment. Eighteen months after the initial surgery the patient is free of disease and in good health. The association of sertoli cell tumor with androgen insensitivity syndrome is discussed and the relevant literature is briefly reviewed.
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2/18. 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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3/18. Androgen receptor gene mutation associated with complete androgen insensitivity syndrome and Sertoli cell adenoma.

    We report a case of Sertoli cell adenoma in complete androgen insensitivity syndrome (CAIS) in a 22-year-old woman. polymerase chain reaction-single strand conformation polymorphism and dna sequencing revealed a single nucleotide substitution on exon 7 of the human androgen receptor (hAR) gene, resulting in a change of CGA (arginine) to CAA (glutamine) in codon 831.
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4/18. PET imaging of sertoli cell tumor in androgen insensitivity syndrome.

    The author presents a case of sertoli cell tumor demonstrated incidentally by F-18 fluorodeoxyglucose positron emission tomography during imaging workup for suspected right-lung malignant neoplasm. This rare sex cord stromal tumor arose from the right gonad of a patient with a history of androgen insufficiency syndrome, also known as testicular feminization syndrome. The relationship of this phenotypic sexual disorder with sertoli cell tumor is briefly reviewed and discussed.
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5/18. Bilateral sertoli cell adenoma and serous cyst in a patient with androgen insensitivity syndrome.

    Thirty-year-old woman with lower abdominal pain was operated due to adnexial mass. cystectomy on right gonad revealed sertoli cell adenoma and simple serous cyst and left gonadal biopsy showed immature testis tissue. Later, laparoscopic left gonadectomy was made. Histopathology of the left gonad was consistent with sertoli cell adenoma.
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6/18. sertoli-leydig cell tumor of the ovary: a rare cause of amenorrhea.

    A 15-year-old girl presented to a primary care clinic with secondary amenorrhea, masculinization, and rapidly increasing abdominal girth. Evaluation revealed a large pelvic mass, which was found at laparotomy to be a bilateral sertoli-leydig cell tumor producing androgens and high levels of alpha-fetoprotein (AFP). The patient underwent bilateral salpingo-oophorectomy. Other pelvic structures were uninvolved. The postoperative course was unremarkable, with testosterone and AFP levels in the normal range. A thorough literature search revealed no cases in which bilateral Sertoli-Leydig cell tumors were associated with high AFP levels. This case was unusual in its pathology, dramatic in its presentation, and valuable in its implications regarding the work-up of amenorrhea in young women.
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7/18. Elevated serum alpha-fetoprotein associated with Sertoli-Leydig cell tumors of the ovary.

    Two young girls are described who presented with amenorrhea, large ovarian masses, elevated serum alpha-fetoprotein (AFP), and signs of androgen excess. Whereas the findings of an ovarian mass and elevated serum AFP typically suggest an ominous diagnosis, ie, endodermal sinus tumor of the ovary, both of these patients were found at laparotomy to have Sertoli-Leydig cell tumors of the ovary. Unilateral oophorectomy rapidly led to undetectable AFP levels in both patients, and resumption of menses.
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8/18. An unusual hormone pattern in a virilized woman affected by sertoli-leydig cell tumor. Report of a case.

    A 24-year-old woman was admitted to hospital because of hirsutism, virilism and amenorrhea, which had appeared 6 months earlier. Endocrinological evaluation showed a slightly elevated serum level of testosterone (1.2 /- 0.05 ng/ml), normal plasma levels of dehydroepiandrosteronesulfate (DHEA-S) (2,070 /- 6 ng/ml), androstenedione (1.8 /- 0.5 ng/ml) and sex hormone-binding globulin (SHBG)(42 /- 3 nM/L); there was normal urinary 17-ketosteroid (17-KS) excretion (11.7 mg/24 h), low urinary estrogen (E) excretion (3 /- 0.4 micrograms/24 h), suppressed basal gonadotropin concentrations (LH 0.9 microUI/ml; FSH 3.2 microUI/ml) and an exaggerated response to the LH-RH test. At laparotomy, a monolateral ovarian tumor was found, which was proved histologically to be a sertoli-leydig cell tumor. After tumor ablation, a regular menstrual cycle followed and progressive reduction of virilism was noted. This was followed within 4 months by complete normalization of LH, FSH, estrogen and progesterone serum levels. The responsiveness to LH-RH also became normalized. Two years after this operation, the patient had a normal pregnancy. This case of virilization in a woman affected by a benign sertoli-leydig cell tumor was primarily characterized by an unusual response of the hypothalamopituitary axis against an endocrinological background of notable alteration of the androgen/estrogen ratio, where the androgens were slightly increased and the estrogens greatly reduced.
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9/18. norethindrone inhibition of testosterone secretion by an ovarian sertoli-leydig cell tumor.

    A woman with severe hyperandrogenemia and virilization was found to have a fall in serum testosterone (T) concentration while taking an oral contraceptive containing norethindrone (500 vs. 164 ng/dL). Subsequent surgical exploration revealed an ovarian sertoli-leydig cell tumor. in vitro, the tumor secreted T (mean, 1.88 ng/mg X 4 h). hCG did not stimulate T secretion. In addition, norethindrone inhibited T secretion (0.33 ng/mg X 4 h). We conclude that norethindrone directly suppressed T production by the sertoli-leydig cell tumor.
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10/18. A testicular tumor resembling the sex cord with annular tubules in a case of the androgen insensitivity syndrome.

    Sex cord tumor with annular tubules (SCTAT) is a distinctive neoplasm with indifferent cells of sex cord derivation in a characteristic arrangement of ring-like tubules. Much attention has been drawn to its association with the peutz-jeghers syndrome (PJS) with reported occurrence of the tumor in the testis of a boy with PJS. The authors present two cases of the androgen insensitivity syndrome (AIS), one of the cases being distinctive in having a large multicystic tumor resembling the SCTAT in the immature gonad. Additionally, the focal areas of the tumor, the large sertoli cells lining the tubules, resembled those of a large cell calcifying sertoli cell tumor (LCCSCT) although no calcific areas were discernible. Although the occurrence of neoplasms like germinomas and tubular adenomas is well known in the AIS, SCTAT has hitherto not been reported in a gonad of the AIS. SCTAT has been placed under an "unclassified sex cord-stromal" category in the world health organization (WHO) classification, yet, opinions are divided as to its origin from a granulosa or Sertoli cell, although an overlap in the histologic features of the two cell categories is to be anticipated in view of their homologous nature. In the case presented, the close resemblance of the tumor cells to the sertoli cells of the uninvolved gonad would further support the concept of a Sertoli line of differentiation of the SCTAT.
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