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      TUMOR DE CELULAS DE SERTOLI Y LEYDIG DEL OVARIO

      case-report

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          Abstract

          Se reporta el caso de una paciente de 16 años con un tumor de células de Sertoli y Leydig del ovario en etapa I tratado con cirugía exclusiva. Además, se realiza una revisión de la literatura sobre este tipo de tumor

          Translated abstract

          We report a sixteen years-old patient with a Sertoli-Leydig cell tumor, stage I, treated with surgery alone. We also present a review of the literature on this type of tumor

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          Most cited references45

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          Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases.

          The clinical and pathological features of 207 ovarian Sertoli-Leydig cell tumors from our consultation and hospital files were reviewed. The patients ranged in age from 2 to 75 (average 25) years. Seventy-five percent of them were 30 years of age or younger and less than 10% were over 50 years of age. One-third of the patients presented because of unequivocal evidence of androgen excess, and an additional 10% had a history suggesting androgen excess; most of the remaining patients complained of abdominal swelling or pain. At operation, 97.5% of the tumors were Stage I, 1.5% were Stage II, and 1% were Stage III. Both ovaries were involved in 1.5% of the cases. The tumors ranged from microscopic to 51 cm in diameter (average 13.5 cm); 15% of them were ruptured. Thirty-eight percent of the tumors were solid, 58% were solid and cystic, and 4% were cystic. The solid tissue was typically lobulated and yellow. On microscopic examination, 11% of the tumors were well differentiated, 54% were of intermediate differentiation, 13% were poorly differentiated, and 22% contained heterologous elements according to the criteria of the World Health Organization; a prominent retiform pattern was present in 15% of them. Follow-up was obtained for 164 patients. The tumor was clinically malignant in 18% of them. The prognosis correlated most meaningfully with the stage and degree of differentiation of the tumor. The high-stage tumors were all clinically malignant. All the well-differentiated tumors were benign, but 11% of those of intermediate differentiation, 59% of the poorly differentiated tumors, and 19% of those with heterologous elements were malignant. In a few cases radiation therapy, chemotherapy, or a combination of the two, in addition to surgical excision, was of benefit in the management of the malignant tumors.
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            Management of early ovarian cancer: germ cell and sex cord-stromal tumors.

            Malignant ovarian germ cell tumors (OGCT) and sex cord-stromal tumors (OSCST), each of which account for less than 5% of all ovarian malignancies, are much less common than epithelial ovarian cancer. In young patients suspected of having an OGCT, laparotomy is initially indicated for both diagnosis and treatment. For most patients, unilateral salpingo-oophorectomy with preservation of the contralateral ovary and the uterus is appropriate. The basis for this surgical approach is retrospective studies that show an equivalent cure rate for patients who undergo unilateral or bilateral adnexectomy. No prospective studies have compared unilateral with bilateral adnexectomy. Surgical staging is also important to determine the extent of disease, to determine prognosis, and to guide postoperative management. If metastatic disease is encountered during initial surgery for OGCT, the same principles of cytoreductive surgery that have been applied to surgically manage advanced epithelial ovarian cancer are recommended, with resection of as much tumor as is technically feasible and safe. For all OGCT patients except those with well-documented stage IA grade 1 pure immature teratoma or stage IA pure dysgerminoma, postoperative chemotherapy is indicated. The current recommended regimen for OGCT is bleomycin, etoposide, and cisplatin--a combination that appears to result in at least a 95% cure rate for stage I disease and at least a 75% cure rate for advanced-stage disease. For patients with metastatic dysgerminoma, chemotherapy, which has the advantage of preserving fertility in the majority of patients, has supplanted radiotherapy as standard treatment. For patients with OSCST, no standard therapy exists. Surgery alone is currently acceptable treatment for all patients with OSCST except those who have metastatic disease or Sertoli-Leydig cell tumors with poor differentiation or heterologous elements. Currently, platinum-based combination chemotherapy is favored for these latter patients, but the activity of such regimens appears only modest.
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              Sertoli-Leydig tumors of the ovary. A clinicopathologic study of 64 intermediate and poorly differentiated neoplasms.

              The clinical and pathologic features of 64 Sertoli-Leydig tumors of the ovary with intermediate and poor differentiation were studied. The neoplasms occurred mainly in young women. Fifty-four percent of the patients presented with clinical evidence of a hormonally active tumor, and 38% were virilized. The remaining 46% had nonspecific symptoms. Sixty-two patients had tumors confined to one ovary at operation (Stage Ia), while only two patients presented with pelvic metastases (Stage III). The prognosis was generally favorable; the 5- and 10-year actuarial survival rates were 92%. Unilateral salpingo-oophorectomy was effective treatment for Stage Ia Sertoli-Leydig tumors in young women. Microscopically, 44 of the neoplasms were of intermediate differentiation and 20 were poorly differentiated. Heterologous elements (mucinous epithelium, striated muscle, cartilage) were present in 16 neoplasms. The pathologic features that correspond with development of metastases were poor differentiation, the presence of heterologous mesenchymal elements, frequent mitotic figures in stromal cells, and rupture of the neoplasm.
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                Author and article information

                Journal
                rchog
                Revista chilena de obstetricia y ginecología
                Rev. chil. obstet. ginecol.
                Sociedad Chilena de Obstetricia y Ginecología (Santiago, , Chile )
                0048-766X
                0717-7526
                2003
                : 68
                : 5
                : 399-406
                Affiliations
                [02] orgnamePontificia Universidad Católica de Chile orgdiv1Unidad de Oncología Ginecológica Chile
                [01] orgnamePontificia Universidad Católica de Chile orgdiv1Deparamento de Obstetricia y Ginecología Chile
                [03] orgnamePontificia Universidad Católica de Chile orgdiv1Departamento de Anatomía Patológica Chile
                Article
                S0717-75262003000500008 S0717-7526(03)06800508
                10.4067/S0717-75262003000500008
                f4c02c42-e133-49f5-b495-7361893418a3

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : September 2003
                : November 2003
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 27, Pages: 8
                Product

                SciELO Chile

                Categories
                Caso Clínico

                and sex cord-stromal tumors,Sertoli-Leydig cell tumor,ovarian neoplasm,Tumor de Sertan y Leydig

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