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[Thecomas and granulosa cell tumors: clinical, morphological and therapeutic aspects].
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[A rare case of bilateral thecoma].
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[Ovarian thecoma. Description of 2 cases].
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[Thecomas (theca cell tumours)].
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Ovarian fibro-thecoma in a 19 years old Sudanese girl. Gynaecological case report.
An unmarried, 19 years old, Sudanese girl developed a firm right ovarian mass which increased in size to reach a diameter of 25 x 25 cm and a weight of 2.8 kg in 8 months time. The patient's condition was associated with amenorrhoea, polycystic ovary and ascites. The ovarian tumour was diagnosed by clinical examination and ultrasound scanning and was identified as a fibrothecoma by histopathology.
Virilizing stromal Leydig cell tumor (Leydig cell-containing thecoma) of the ovary in pregnancy. A case report with extensive immunohistochemical investigation of the tumor cells.
The rare case of a stromal Leydig cell tumor of the ovary occurring in a 21-year-old woman who developed signs of virilization during pregnancy is reported. Serum androgen levels were markedly elevated. At cesarean section, a slightly hypotrophic, but otherwise normal, female infant was delivered and a tumor of the right ovary measuring 12 cm in maximum diameter was resected. Histologic examination revealed a sex cord-stromal tumor consisting of spindle-shaped, thecomatous cells and a large number of loosely scattered clusters of large polygonal cells with abundant eosinophilic cytoplasm. Both types of tumor cells were strongly immunoreactive for vimentin, but exhibited no proliferative activity and no overexpression of p53 protein. A few of the polygonal cells contained typical crystalloids of Reinke. Cellular atypia was not a prominent feature, and a diagnosis of benign stromal Leydig cell tumor was established. As expected, 20 months after diagnosis the patient exhibits no signs of recurrence or dissemination. To the best of our knowledge this is only the second case of a stromal Leydig cell tumor occurring in pregnancy to be described.
Bilateral non functionning thecoma of the ovary in epileptic children under anticonvulsant therapy.
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Cytomegalovirus infection of ovarian thecoma.
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[Ovarian thecoma associated with adrenocarcinoma of the endometrium. Biochemical study].
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[Ovarian tumor associated with a vegetating cyst and a granulo-thecoma in a young woman].
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Is loss of heterozygosity at 9q22.3 (PTCH gene) and 19p13.3 (STK11 gene) involved in the pathogenesis of ovarian stromal tumors?
Some ovarian fibromas and rare fibrosarcomas are associated with Gorlin syndrome, which is caused by mutation in the human homologue of Drosophila patched gene (PTCH), localized on chromosome 9q22.3. The relationship between PTCH gene and sporadic ovarian tumors in the thecoma-fibroma group has not been well characterized. On the other hand, we have recently described loss of heterozygosity (LOH) at 19p13.3 in 2 sporadic fibromas with sex-cord elements. We have analyzed DNA from 8 fibromas, 6 cellular fibromas, 2 fibrothecomas, 9 luteinized thecomas, and 2 fibrosarcomas of the ovary for LOH at 9q22.3 and 19p13.3, using polymerase chain reaction amplification for 10 microsatellite markers. LOH at 9q22.3 was detected in 4 (67%) of 6 cellular fibromas, with the highest frequency at microsatellite marker D9S15, which localizes proximal to the PTCH gene. Of 9 luteinized thecomas, 2 (22%) also exhibited LOH at 9q22.3 with 3 microsatellite markers other than D9S15. Allelic losses were not detected in any fibroma, fibrothecoma, or fibrosarcoma. LOH at 19p13.3 was found in 2 (25%) of 8 fibromas, 3 (50%) of 6 cellular fibromas, and 1 (11%) of 9 luteinized thecomas. None of the 2 fibrothecomas or 2 fibrosarcomas showed LOH at 19p13.3. LOH at both 9p22.3 and 19p13.3 was observed in 3 (50%) of 6 cellular fibromas, but not in luteinized thecomas. The results indicate that (1) LOH at both PTCH gene and STK11 gene is relatively frequent in cellular fibromas; (2) approximately a quarter of luteinized thecomas exhibited LOH of the PTCH gene; in both neoplasms, cellular fibromas and luteinized thecomas, LOH may play a role in their pathogenesis; and (3) sporadic cellular fibromas may arise through similar genetic pathways as cases of Gorlin syndrome.
Sclerosing stromal tumor of the ovary: an ultrastructural and immunohistochemical analysis with histogenetic considerations.
Sclerosing stromal tumors are rare, benign ovarian neoplasms of unknown etiology and histogenesis. Three sclerosing stromal tumors were evaluated by immunohistochemistry and electron microscopy and were compared to two thecomas and nonneoplastic ovarian mesenchymal tissue. The sclerosing stromal tumors and thecomas were positive for muscle-specific actin; immunoreactivity was intense in the cellular areas of the sclerosing stromal tumors and focal in the thecomas. This antigen was expressed in nonneoplastic stroma predominantly in a perifollicular (theca externa) distribution. Two sclerosing stromal tumors and both thecomas were vimentin positive. Desmin was present in nonvascular cells in one of each tumor type. Expression of vimentin diffusely and of desmin focally was present in nonneoplastic cortical stroma and surrounding follicles. All specimens were nonreactive for cytokeratin. Electron microscopy supported differentiation toward smooth muscle in the sclerosing stromal tumors but not in the thecomas. Such differentiation included aggregates of cytoplasmic filaments with interspersed dense bodies, pinocytotic vesicles, and basal lamina. Delicate, long processes interconnected cells, often with primitive junctions, in the hypocellular foci. Cytoplasmic lipid, which was present in the thecomas, was not well developed in the sclerosing stromal tumors. It is proposed that a population of muscle-specific actin-positive elements exists in the theca externa--the perifollicular myoid stromal cell--and that sclerosing stromal tumors may originate from them. Sclerosing stromal tumors and thecomas share many antigenic determinants and morphologic features and thus are probably closely related entities.
[Ovarian fibrothecal tumors. Apropos of 12 cases].
OBJECTIVE: To identify diagnosis, therapeutic and histological particularities of ovarian fibro-thecomas. STUDY DESIGN: A retrospective study of 12 patients who underwent surgical treatment for ovarian fibro-thecoma between January 1994 and December 1999 was reported. Clinical, ultRasonographic, tumour marker, therapeutic and histologic data were analysed. RESULTS: Mean age of patients was 46.83 years. Three patients were in perimenopausal period and six were menopausal. Ultrasonographic findings were: ovarian echogenic tumor in six cases, hypoechogenic tumor in three cases, mixed tumor in three cases and anechogenic tumour in one case. CA 125 level measured in 11 cases was normal. Nine patients underwent laparotomy. Three patients underwent laparoscopy, however, one was converted to laparotomy due to a suspected tumour. A conservative treatment was performed for three patients and radical treatment for nine patients. Histological findings were eight fibromas and five fibro-thecomas. CONCLUSION: Ovarian fibro-thecoma is uncommon. These lesions occur often in perimenopausal and menopausal patients. Clinical, ultrasonographic and tumour marker data remains the best preoperative approach currently available for ovarian tumours. However, the diagnosis remains histological. Tumorectomy is well indicated for young patients, however radical treatment is indicated for perimenopausal and menopausal patients.
Immunohistochemical study of 3 beta-hydroxysteroid dehydrogenase in sex cord-stromal tumors of the ovary.
3 beta-Hydroxysteroid dehydrogenase (3 beta-HSD), which converts pregnenolone to progesterone, was localized immunohistochemically in 18 thecomas, 23 fibromas, 5 granulosa-cell tumors, 5 sclerosing stromal tumors, and 2 steroid-cell tumors. Immunohistochemical study of estrogen, progesterone, and testosterone was also performed in serial sections of thecomas and fibromas. In thecomas, immunoreactivity of 3 beta-HSD was observed only in luteinized theca cells and thecomatous tumor cells with abundant pale to vacuolated cytoplasm but not in spindled tumor cells and thecomatous tumor cells with small to moderate amounts of pale to vacuolated cytoplasm. Immunoreactivity of steroids was not observed in thecomas except for testosterone immunoreactivity in one case. No immunoreactivity of steroids or the enzyme was present in fibromas. No tumor cells were positive for 3 beta-HSD in any of the cases of granulosa-cell tumor examined. Immunoreactivity of 3 beta-HSD was present in cells in steroid-cell tumors and polygonal tumor cells with prominent cytoplasmic vacuoles in two cases of sclerosing stromal tumor. Thus, 3 beta-HSD can be a good immunohistochemical marker of steroidogenesis in functioning ovarian neoplasms.
Sex steroid biosynthesis enzymes in ovarian sex-cord stromal tumors.
Previous studies on neoplastic and hyperplastic ovarian lesions using paraffin-embedded material have demonstrated immunolocalization of sex steroid biosynthetic enzymes (SSBEs): P-450 side chain cleavage (P-450 SCC), which converts cholesterol to pregnenolone; 3 beta-hydroxysteroid dehydrogenase (3 beta-HSD), which converts pregnenolone to progesterone; P-450 17 alpha-hydroxylase and lyase (P-450 17A), which convert progesterone to 17 alpha-hydroxyprogesterone and 4-androstene-3,17-dione; and P-450 aromatase (P-450 AR), which converts 4-androstene-3,17-dione to estradiol. To investigate the utility of immunohistochemical staining for SSBEs, we studied a series of 45 sex cord-stromal tumors of the ovary. P-450 SCC was present in 9 of 11 Sertoli-stromal cell tumors, 3 of 12 granulosa cell tumors, 2 of 7 thecomas, and 1 of 1 stromal luteomas; 3 beta-HSD was present in 5 of 11 Sertoli-stromal cell tumors, 2 of 12 granulosa cell tumors, 2 of 7 thecomas, and 1 of 1 stromal luteoma; P-450 17A was present in 5 of 11 Sertoli-stromal cell tumors, 2 of 12 granulosa cell tumors, 2 of 6 thecomas, and 1 of 1 stromal luteomas; P-450 AR was present in 6 of 11 Sertoli-stromal cell tumors, 2 of 12 granulosa cell tumors, none of 7 thecomas, and 1 of 1 stromal luteoma. SSBEs were not present in 12 fibromas, one sclerosing stromal tumor, and one myxoma. Five of 45 patients with sex cord-stromal tumors showed androgenic effects; 4 of 11 patients with Sertoli-stromal cell tumors and the patient with a stromal luteoma. These five sex cord-stromal tumors contained P-450 SCC, and three of four of the Sertoli-stromal cell tumors contained 3 beta-HSD, P-450 17A, and P-450 AR. Concurrent endometrial histology was available in 25 of 45 sex cord-stromal tumor patients. None of the five sex cord-stromal tumors arising in patients with endometria that showed hyperplasia or adenocarcinoma showed immunoreactivity for SSBEs. Eight patients' endometria were unremarkable, but their sex cord-stromal tumor contained SSBEs. SSBEs were present in areas showing Leydig cell, Sertoli cell, or steroid cell differentiation or luteinized areas; however, the results did not significantly add to the histologic classification of sex-cord stromal tumors. Androgenic hormonal effects could always be explained by synthesis of hormones by SSBEs present in the patient's sex cord-stromal tumor.(ABSTRACT TRUNCATED AT 400 WORDS)
Sex cord-stromal tumours of the ovary- a 25 year review.
We present a retrospective study of granulosa cell tumours and thecomas encountered in one hospital between 1970 and 1995. There were 17 granulosa cell tumours and 17 thecomas. The size of the granulosa cell tumours varied from 3 cm to 30 cm in diameter, and no correlation was found between size and evidence of invasion. There was also no correlation between either mitotic counts or histological pattern and evidence of invasion. Evidence of oestrogen production was found in 12/17 granulosa cell tumours (two with endometrial carcinoma and 10 with endometrial hyperplasia) and in 9/17 of the thecomas (two with endometrial carcinoma and seven with endometrial hyperplasia). Thecomas are regarded as benign tumours, but granulosa cell tumours are characterised by a long natural history with a significant capacity to recur years after an apparent clinical cure. It is therefore important that patients with these tumours are followed up indefinitely.
[Recurrent theca cell tumor].
Thecomas are considered to be benign ovarian tumours. Whether there are malignant thecomas, is a subject of controversy in literature. This contribution describes a case of recurrent thecoma with a clinically malignant course and histological signs of malignant dedifferentiation. The particular diagnostic and therapeutic consequences of this rare disease are discussed with emphasis on the need for radical surgical intervention.