MR findings of primary ovarian granulosa cell tumor with focus on the differentiation with other ovarian sex cord-stromal tumors.

BACKGROUND: To describe magnetic resonance imaging (MRI) features of ovarian granulosa cell tumors (OGCTs) and compare with other sex cord-stromal tumors (OSCs) in ovary. METHODS: MR findings of 18 patients with surgically confirmed ovarian granulosa cell tumor were retrospectively reviewed by two radiologists with consensus reading. All MR examinations were prospectively performed within one month. Clinical and imaging characteristics of OGCTs were evaluated and compared with OSCs (control group). RESULTS: In 18 patients, 20 ovarian granulosa cell tumors were detected on MRI. Sixteen tumors appeared as solid or mostly solid mass (16/20), while 4 tumors as cystic mass. Pathological pelvic fluid was detected in 1 OGCT (1/18) and 11 OSCs (11/34) (p = 0.031).On T2 weighted imaging (T2WI), most of OGCTs displayed hyperintense signal and mixed signal (19/20); on T1 weighted imaging (T1WI), 11 OGCTs (11/20) displayed similar signal as on T2WI imaging. The lesion signal between OGCT and OSC differed significantly on both T1WI (p = 0.017) and T2WI (p = 0.002). Tumoral bleeding was detected in 6 OGCTs on MRI. On diffusion weighted imaging (DWI) images, OGCTs mostly appeared as high signal (16/20). Average apparent diffusion coefficient (ADC) value derived from DWI images in the OGCT group (0.84 ± 0.26× 10- 3 mm2/s was less than the control group (1.22 ± 0.47 × 10- 3 mm2/s) with statistical difference (p = 0.002). CONCLUSIONS: MRI could provide important information in OGCT diagnosis. ADC value might be useful in differentiating OGCT from OSC.

A Rare Presentation of Paraovarian Sclerosing Stromal Tumor with High Mitotic Activity.

BACKGROUND: Sclerosing stromal tumor is an extremely rare type of benign ovarian sex cord stromal tumor. CASE: The benign characteristic of this tumor is well known but we present an uncommon case of paraovarian sclerosing stromal tumor with high mitotic activity. RESULTS AND CONCLUSION: Despite this potential malignancy, our patient was treated successfully with enucleation only.

Tumor ovárico no epitelial: tumor de Sertoli-Leydig / Non-epithelial ovarian cancer: Sertoli-Leydig cell tumor

Los tumores de Sertoli-Leydig son un tipo infrecuente de tumor de ovario de estirpe no epitelial; concretamente, pertenecen al grupo de los tumores de los cordones sexuales-estroma, con una incidencia aproximada de un 2-5% de todas las neoplasias ováricas malignas. Presentamos el caso de una paciente de 42 años intervenida de un tumor de Sertoli con focos sarcomatoides. El diagnóstico de estos tumores es difícil de establecer prequirúrgicamente, y debido a la escasez de publicaciones, existe mucha controversia acerca de la estadificación quirúrgica idónea y sobre la conveniencia de realizar o no tratamiento adyuvante (AU)

Sertoli-Leydig cell tumors are a rare type of non-epithelial ovarian cancer; these tumors belong to the group of sex cord-stromal tumors and account for approximately 2-5% of all malignant ovarian tumors. We report the case of a 42-year-old woman who underwent surgery for a Sertoli tumor with sarcomatoid foci. Diagnosis of these tumors is difficult before surgery and, due to the scarcity of publications on the topic, there is controversy about the appropriate surgical staging and the role of adjuvant therapy in the management of this entity (AU)

Steroidogenic acute regulatory protein is a useful marker for Leydig cells and sex-cord stromal tumors.

Steroidogenic acute regulatory (StAR) protein is a rate-limiting protein, which is essential for transporting cholesterol into the mitochondria for steroidogenesis. StAR protein could be a marker for steroidogenic tissues. In this study, we investigated StAR protein levels in sex-cord stromal tumors (SCSTs) including 31 adult granulosa cell tumors, 3 juvenile granulosa cell tumors, 10 fibrothecomas, 2 luteinized thecomas, 4 Sertoli-Leydig cell tumors (SLCTs), 4 sclerosing stromal tumor and 3 Leydig cell tumors (LCTs), and 219 non-SCSTs. SCSTs were used for immunohistochemical staining of StAR protein, α-inhibin, calretinin, and CD99. All the 3 LCTs (100%) strongly stained for StAR protein; 30 of the 31 adult granulosa cell tumors (96%) showed focal staining of StAR protein; all the 10 fibrothecomas and 4 sclerosing stromal tumors (100%) were negative for StAR protein staining; StAR protein stained in Leydig cells but not in Sertoli cells in the 4 SLCTs. All the non-SCSTs were negative for StAR protein except for tumor cells in 4 adrenocortical adenomas and 2 adrenocortical carcinomas. Results of the study indicate that StAR protein is a useful marker for differential diagnosis of SCSTs. It is sensitive and specific for Leydig cells in tumors containing this component (LCT, SLCT), and can express focally in granulosa cell tumors. It is negative for Sertoli cells and nonluteinized theca cells.

Expression of adrenomedullin in human ovaries, ovarian sex cord-stromal tumors and cultured granulosa-luteal cells.

The aim of the present study was to characterise the expression pattern of the multifunctional vasoactive peptide adrenomedullin (ADM) in human ovarian tumors, and to find hormonal regulators of ADM expression in human ovaries. The expression of ADM messenger RNA (mRNA) was higher in granulosa cell tumors than in fibrothecomas and normal ovaries, as analysed by Northern blots. In normal ovaries, ADM immunoreactivity was localised in both granulosa and thecal cells. Eight of the 90 granulosa cell tumors (9%) showed moderate and 53 (59%) weak ADM immunoreactivity, whereas 27% (11/41) of the fibrothecomas displayed weak ADM staining. FSH, protein kinase A activator (Bu)(2)cAMP, prostaglandin E(2) (PGE(2)), activin A and the broad protein kinase regulator staurosporine decreased ADM mRNA accumulation in cultured granulosa-luteal cells time- and dose-dependently. FSH, (Bu)(2)cAMP and PGE(2) increased progesterone secretion and the accumulation of the steroidogenic acute regulatory protein mRNA in these cells. In conclusion, ADM is expressed in normal human ovaries and sex cord-stromal tumors, particularly in those of granulosa cell origin. FSH, PGE(2,) (Bu)(2)cAMP and activin A suppress ADM gene expression in granulosa-luteal cells. Expression of ADM in human ovaries and its hormonal regulation in granulosa cells suggests a paracrine role for ADM in ovarian function.

Bcl-2 and MALT1 Genes are not involved in the oncogenesis of uterine tumors resembling ovarian sex cord tumors.

Uterine tumors resembling ovarian sex cord tumors (UTROSCT) are rare entities. They were described by Clement and Scully in 1976 who classified them into groups I and II. Group I comprises typical endometrial stromal neoplasms with focal areas resembling ovarian sex cord elements and group II are predominantly or completely composed of ovarian sex cord-like elements. We report a case of UTROSCT type II with cytogenetic analysis. The tumor occurred in a 76-year-old woman who presented with vaginal bleeding. The tumor was lobulated, firm, yellow and histologically composed of sex cord-like elements. Tumor cells expressed vimentin, CD10, CD99 and alpha-actin. Cytogenetic analysis in a previously reported case detected translocation t(4;18)(q21.1;q21.3) in the majority of cells. Bcl-2 and MALT1 genes are located at or near the translocation breakpoints, and the aim of this study was to determine whether these genes were involved in chromosomal translocation or tumorigenesis. We did not find IgH translocation or the most common MALT translocations. Bcl-2 was also not involved in this oncogenesis.

The power Doppler velocity index, pulsatility index, and resistive index can assist in making a differential diagnosis of primary ovarian carcinoma and Krukenberg tumors: a preliminary study.

OBJECTIVE: The aim of this study was to compare the effectiveness of transvaginal power Doppler sonography with spectral Doppler analysis as an aid in preoperatively distinguishing primary ovarian carcinoma and metastatic carcinoma to the ovary (Krukenberg tumors). METHODS: Fifty women with ovarian disease were preoperatively examined with transvaginal power Doppler sonography. Six basic parameters were measured, including intratumoral peak systolic velocity, end-diastolic velocity, time-averaged maximum velocity, pulsatility index (PI), resistive index (RI), and velocity index (VeI). Blood flow analyses were detectable in all patients. Twelve patients with metastatic carcinoma to the ovary were classified as group 1; 38 patients with primary ovarian carcinoma were classified as group 2. Comparison of intratumoral blood flow analyses between the two groups was performed. RESULTS: The PI, RI, and VeI were significantly lower in patients with metastatic carcinoma to the ovary than those with primary ovarian carcinoma (P < .05). There were no significant differences in the peak systolic velocity (P = .871), end-diastolic velocity (P = .508), and time-averaged maximum velocity (P = .850) between the two groups. CONCLUSIONS: Transvaginal power Doppler sonography with spectral Doppler analysis is an effective method in evaluating intratumoral blood flow of Krukenberg tumors. Low impedance (PI, RI, and VeI) might assist us in making differential diagnoses between primary ovarian carcinoma and Krukenberg tumors according to our preliminary results.

Expression of betaglycan, an inhibin coreceptor, in normal human ovaries and ovarian sex cord-stromal tumors and its regulation in cultured human granulosa-luteal cells.

Activins and inhibins are often antagonistic in the regulation of ovarian function. TGFbeta type III receptor, betaglycan, has been identified as a coreceptor to enhance the binding of inhibins to activin type II receptor and thus to prevent the binding of activins to their receptor. In this study we characterized the expression and regulation pattern of betaglycan gene in normal ovaries and sex cord-stromal tumors and in cultured human granulosa-luteal cells from women undergoing in vitro fertilization. Expression of betaglycan mRNA was detected by RT-PCR or Northern blotting in normal ovarian granulosa, thecal, and stroma cells as well as in granulosa-luteal cells. Immunohistochemical analysis revealed positive staining for betaglycan in antral and preovulatory follicular granulosa and thecal cells and in corpora lutea of normal ovaries. Furthermore, betaglycan expression was detected in the vast majority of granulosa cell tumors, thecomas, and fibromas, with weaker staining in granulosa cell tumors compared with fibrothecomas. In cultured granulosa-luteal cells, FSH and LH treatment increased dose-dependently the accumulation of betaglycan mRNA, as did the protein kinase A activator dibutyryl cAMP and the protein kinase C inhibitor staurosporine. In contrast, the protein kinase C activator 12-O-tetradecanoyl phorbol 13-acetate had no significant effect on betaglycan mRNA levels. Treatment with prostaglandin E(2) and with its receptor EP2 subtype agonist butaprost increased betaglycan mRNA accumulation and progesterone secretion dose- and time-dependently. In summary, betaglycan gene is expressed in normal human ovarian steroidogenic cells and sex cord-stromal ovarian tumors. The accumulation of its mRNA in cultured granulosa-luteal cells is up-regulated by gonadotropins and prostaglandin E(2), probably via the protein kinase A pathway. The specific expression and regulation pattern of betaglycan gene may be related to the functional antagonism of inhibins to activin signal transduction in human ovaries.