| Sertoli-Leydig cell tumors (SLCT) constitute only 1-0.5% of all primary ovarian neoplasms. We report a SLCT in a postmenopausal woman aged 69 years. The physical examination revealed severe hirsutism. Basal hormonal evaluation showed high plasma testosterone and estradiol values, with suppressed plasma gonadotropins. Computer tomograph scan revealed a right ovarian tumor mass of 4,3/3 cm, confirming an androgen secreting ovarian tumor. The histopathological and immunocytochemical examination established the diagnosis of well differentiated sertoli-leydig cell tumor. The tumor was positive for cytokeratin KL 1 and S-100 protein and, in isolated tumor cells, positive for alpha-fetoprotein. Postsurgical evolution was favorable; controls after 6 months and 3,5 years showed marked reduction of hirsutism, normal plasma testosterone values and gonadotropins in normal postmenopausal range. We discuss the complex aspects of etiology and pathogenesis, the clinical and hormonal settings, the role of immunocytochemical markers in diagnosis, as well as the therapy and the prognostic features of this ovarian tumor. ( info) |
12/66. Sertoli Leydig cell tumour of ovary with heterologous element--a case report. A 16 years old female who presented with abdominal swelling and secondary amenorrhoea was subsequently diagnosed as Sertoli Leydig cell tumour of ovary revealing intermediate differentiation with heterologous element. The case is reported in view of its rarity. ( info) |
13/66. Sertoli Leydig cell tumor with heterologous gastrointestinal epithelium: a case report. Sertoli Leydig cell tumour (SLCT) of the ovary with the presence of heterologous elements is very unusual. A case of SLCT with virilizing symptoms and androgenic endocrinological profile is presented. The tumor showed presence of heterologous element in the form of mucinous epithelium of the gastrointestinal type. ( info) |
| An extremely rare case of a postmenopausal patient with an ovarian Sertoli-Leydig cell tumour and a coexistent vaginal angiomyxoma is reported. A 71-year-old patient was admitted complaining of abdominal distension. A thorough diagnostic evaluation revealed a large tumour of the right ovary, and an oval-shaped greyish-white polypoid vaginal lesion. Total hysterectomy with bilateral salpingooophorectomy and lymph node sampling was performed, followed by excision of the vaginal lesion. Histological examination showed a Sertoli-Leydig cell tumour of the right ovary, and a vaginal angiomyxoma. Twenty-six months after the operation the patient is well with no signs of recurrence. To the best of our knowledge, no case of coexistence of an ovarian Sertoli-Leydig cell tumour with a myxoma has been previously reported. ( info) |
| Sertoli-Leydig cell tumors of the ovary are rare diseases that occur primarily in young women. The majority of these tumors are unilaterally localized, and conservative surgery is sufficient. However, these tumors exhibit a variety of histological patterns, which are significant prognostic factors. To date, no standard therapy exists. Here we report 4 cases of Sertoli-Leydig cell tumors of the ovary. One patient whose tumor was a poorly differentiated sertoli-leydig cell tumor with mesenchymal heterologous elements received adjuvant chemotherapy postoperatively but died of disease 2.5 years after surgery. The other 3 patients remained free of disease during follow-up. Conservative surgery is an appropriate treatment for young patients with Sertoli-Leydig cell tumors. Those who have poor prognostic factors may need adjuvant chemotherapy with a combination of bleomycin, etoposide and cisplatin. ( info) |
16/66. 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. ( info) |
17/66. 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. ( info) |
| Sertoli-Leydig cell tumors are uncommon tumors of ovary accounting for about 1% of sex cord stromal tumors. They constitute between 0.1% to 0.5% of all the primary ovarian neoplasms. The majority of reported cases are moderately or poorly differentiated combined Sertoli-Leydig cell tumors. A rare case of sertoli-leydig cell tumor with heterologous element in a 14 year girl is described. The heterologous component comprised smooth muscle, an uncommon element, that was seen in 60 to 70% of the tumor area. The epithelial element of the tumor consisted of solid, tubular and foci of retiform pattern. Both these features imply a poor prognosis. ( info) |
19/66. Second and third malignant solid tumor in a girl with ovarian Sertoli-Leydig tumor. We report a Sertoli-Leydig cell (SLC) tumor of the right ovary in a 10-year-old girl, which was dealt with surgical removal. Three months after resection, she presented with a new episode of acute abdomen because of an abdominal mass, which histologically was compatible with an undifferentiated embryonal rhabdomyosarcoma. Chemotherapy, according to SIOP-??? 89 protocol, was administered additionally to radiotherapy (3,960 cGy). Three years after completing treatment, the patient developed a painful swelling at her left upper arm. The diagnosis was Ewing sarcoma of the humerus, which was confirmed by identification of the typical 11; 22 translocation on cytogenetic and molecular analysis of the tumor tissue. The patient died 14 months from Ewing diagnosis due to progressive disease. ( info) |
| sertoli-leydig cell tumor belongs to the group of sex cord-stromal tumors of the ovary. These neoplasms account for less than 0.5% of all ovarian tumors and are more often encountered in young women between the ages of 20 and 30 years who usually become virilized. We described an unusual case of sertoli-leydig cell tumor in a postmenopausal women who presented with a solid right pelvic mass, a large amount of ascites, and laboratory tests revealing an elevated CA125, all suggesting a pelvic malignancy. Although five similar cases of postmenopausal women with sertoli-leydig cell tumor of ovary have been reported in the literature, we believe that this is an useful addition to the literature. ( info) |