Giant Ovarian Thecoma Associated with Meigs Syndrome: A Striking Case.

Introduction: Meigs syndrome consists of the presence of a benign ovarian tumor, ascites and pleural effusion, and the latter two subdued after surgical excision of the ovarian tumor. Elevated Ca-125 in this context is confusing and is reported only in a handful of cases. A rare but striking case with the above features is presented herein. Case Presentation: A 46-year-old woman with a giant pelvic/abdominal mass originating from her right adnexa, ascites and pleural effusion, with elevated Ca-125 (938 IU/mL) was treated with the presumptive diagnosis of stage IV ovarian cancer. Imaging modalities showed a 22 cm solid adnexal mass and the patient underwent total abdominal hysterectomy and bilateral salpigooophorectomy, omentectomy and drainage of 4L of ascetic fluid. Surprisingly, final histopathology was negative for malignancy, characterizing the primary tumor as ovarian thecoma. Ascites and pleural effusion resolved by the seventh postoperative day, setting the diagnosis of Meigs syndrome. Discussion: Meigs syndrome accounts for 1% of all ovarian tumors, however it should be considered in the differential diagnosis when clinicians come across the classic triad of the syndrome, even when Ca-125 is elevated. These patients have normal life expectancy with meticulous management, while pathophysiology of this condition remains uncertain in various points.

Adolescent ovarian thecoma presenting as progressive hyperandrogenism: case report and review of the literature.

Hyperandrogenism is frequent and under investigated in adolescent girls. A 15-year-6-month-old French girl presented with oligomenorrhea and slowly progressing virilization 2 years post-menarche. Medical history revealed prenatal pesticide exposure through maternal professional activity and recurrent premature thelarche. Severe hirsutism, mild facial acne and clitoromegaly were noted. Serum androgens (testosterone: 94 ng/dL, 4-androstenedione: 8.23 ng/mL) were high and non-classic 21-hydroxylase deficiency was excluded. Pelvic ultrasonography showed a left ovarian mass, confirmed by computed tomography scan. Tumor markers were negative. Laparoscopic surgery was performed. The pathological diagnosis was benign luteinized thecoma. Postoperatively, the menstrual cycle and serum androgens became normal and hirsutism slowly improved. Hyperandrogenism 2 years after menarche should be systematically investigated, even if slowly progressive, since it may be a symptom of a rare virilizing ovarian tumor, like thecoma.

Granulosa Cell Tumors of the Ovary With Prominent Thecoma-like Foci: A Report of 16 Cases Emphasizing the Ongoing Utility of the Reticulin Stain in the Modern Era.

Sixteen adult granulosa cell tumors which had conspicuous zones of cells with pale cytoplasm imparting a resemblance to thecoma are reported. The neoplasms occurred in patients from 38 to 86 yr of age, the majority being over 55 yr of age. Ten tumors were incidental findings, the remainder being associated with symptoms or signs related to an adnexal mass. All the tumors were unilateral, typically small, usually under 5 cm, with only 3 being larger. With 1 exception they were uniformly solid and were typically entirely or focally yellow on sectioning. Microscopic examination typically showed a nodular pattern of growth constituted by cells with moderate to abundant pale cytoplasm; the cells resembled those seen in most thecomas. The nodules occasionally became confluent and focally a diffuse pattern was seen. Typical foci of adult granulosa cell neoplasia in the form of foci of conspicuous epithelial differentiation were absent or rare in most cases but were seen in subtle form in 6 cases and overtly in 3. A few tumors had other features seen in some thecomas, hyaline plaques, sclerosis, and calcification. Reticulin stains were examined in 13 cases and showed that the thecoma-like foci exhibited a dearth of reticulum indicating that those areas were predominantly of granulosa cell nature. Most adult granulosa cell tumors have cells with scant cytoplasm; occasional tumors have abundant eosinophilic cytoplasm, so-called luteinized adult granulosa cell tumors. That some granulosa cell tumors have the cytoplasmic features described herein has occasionally been noted but the resemblance to thecoma has not been emphasized to the best of our knowledge and in the past such tumors may have been misdiagnosed as thecoma, the referral diagnosis in 6 of our cases. A reticulin stain is of crucial aid in indicating the epithelial nature of the thecoma-like foci in these cases. Given the small size of the majority of the tumors the distinction between a small adult granulosa cell tumor and thecoma does not have significant prognostic or therapeutic implications in most cases but awareness of this feature of a small subset of adult granulosa cell tumors is warranted. Our findings have import to the diagnosis of thecoma which is uncommon if strict criteria, including exclusion of granulosa tumors of the type described, are used.

FOXL2 Mutation Analysis of Ovarian Sex Cord-Stromal Tumors: Genotype-Phenotype Correlation With Diagnostic Considerations.

Correlation of FOXL2 mutation status with morphologic features and reticulin staining patterns was performed in a comprehensive single-institutional cohort of ovarian sex cord-stromal tumors. Fifty-one cases were included, 35 of which were morphologically diagnosed as adult granulosa cell tumor, 4 as Sertoli-Leydig cell tumor, 11 as fibroma/fibrothecoma and 1 as a thecoma. Of the adult granulosa cell tumors, 31 (88.6%) harbored FOXL2 mutation. Abundant pale cytoplasm was seen in 51.6% (16/31) of FOXL2 mutated tumors, compared with 6.7% (1/15) among FOXL2 wild type tumors (P=0.003). Nearly half of FOXL2 negative tumors showed individual pericellular reticulin staining pattern, while none of the FOXL2 positive cases demonstrated this feature (P=0.0001). Nested reticulin pattern was observed in 67.7% of FOXL2 positive tumors, compared with 20% of FOXL2 negative cases (P=0.004). Indeterminate reticulin staining pattern was seen in nearly one third of cases in both groups. Nested reticulin pattern was 87.5% specific and 67.7% sensitive for FOXL2 mutation, while individual reticulin pattern was 100% specific for absence of FOXL2 mutation. No statistical significance was observed between the 2 groups in tumor size, mitotic activity, nuclear atypia, and nuclear grooves. Follow-up was available for 44 patients ranging from 0.3 to 259 months (mean: 67.5 mo). Two patients developed recurrence, both of them harbored FOXL2 mutation. In conclusion, the pathology diagnosis of sex cord-stromal tumors continues to be difficult, and reticulin staining remains a valuable tool as an initial step in the diagnostic work-up. Individual pericellular reticulin pattern essentially rules out adult granulosa cell tumor, while cases with indeterminate or nested patterns can be subjected to FOXL2 mutation testing to aid the diagnosis.

FOXL2 Mutation Status in Granulosa Theca Cell Tumors of the Ovary.

Ovarian sex-cord stromal tumors that have between 10% and 50% granulosa cells in a prominent fibrothecomatous background have been referred to as granulosa theca cell tumors or mixed granulosa theca cell tumors. The classification and prognosis of these tumors is not clear. Most adult granulosa cell tumors of the ovary harbor a mutation in the FOXL2 gene, whereas fibromas and thecomas lack this mutation. The aim of our study was to assess the FOXL2 mutation status of ovarian granulosa theca cell tumors and to correlate the mutation status with morphologic and clinical characteristics. A FOXL2 mutation was detected in 6 of 12 (50%) granulosa theca cell tumors. Tumors with higher cellularity of granulosa cells were more likely to harbor a FOXL2 mutation as were tumors in which the granulosa cells formed large lobules. No conclusions could be drawn regarding the clinical and prognostic significance of the presence of a mutation given the small number of cases and limited clinical follow-up. Our study shows that half of granulosa theca cell tumors harbor the same FOXL2 mutation that characterizes adult granulosa cell tumors but there is no outcome evidence to guide whether mutation status should alter the classification of the tumor or the management of the patient.

Contribution of ultrasound in the study of ovarian fibrothecomas: a series of 47 cases.

INTRODUCTION: Ovarian fibro-thecoma are rare presenting 1 to 4, 7%of ovarian organictumors. These tumors are of stromal origin and contain varied proportion of fusiform connective tissue cells and theca cells. They mainly affect menopausal or perimenopausal women. This tumor is benign in most cases and may be responsible for hormone secretion. OBJECTIVES: Study the epidemiological and clinical data of patients with ovarian fibro-thecoma, analyze ultrasonographic characteristics of these tumors,and evaluate the sensitivity and specificity of pelvic ultrasound in ovarian fibro-thecoma approach. METHODS: A retrospective study of 47 patients who underwent surgical treatment for ovarian fibro-thecoma was performed. Data were collected in our department of gynecologyand obstetrics A within Charles Nicole hospital in Tunis, over a period of 18 years between January 1994 and December 2012. For each of our observations, we analyzed the clinical and para-clinical data, including U.S. characteristics and available MRI data with confrontation to the final histological results. RESULTS: The average age of patients was 45.2 years. The average gravidity was 4 and the mean parity was 3. . Thirty-eight of our patients were postmenopausal (80.85%). Ovarian tumor was discovered incidentally in 11 cases and on the occasion of functional symptoms in 36 cases including pelvic pain in 18 cases. Physical examination revealed a pelvic mass in 17 patients and pelvic-abdominal in 14 patients. All patients underwent a pelvic ultrasound. . Ultrasound identified 49 tumors (2 cases of bilateral tumors). Average size of tumors was 10, 05 cm (4 to 30 cm). ) . Ovarian tumor was echogenic in 9 cases (18.36%), hypoechoic in 14 cases (28.47%), mixed in 14 cases (28.47%) and anechoic in 12 cases (24.49%). The tumor was found to be solid in 27 cases (55.1%); cystic in 8 cases (16.3%) and solido cystic in 14 cases (28.6%).It was compartmentalized in 10 cases. Extra cystic vegetations were found in 2 patients. The tumor was nonvascularized at color Doppler in 47 cases (95.9%) and slightly vascularized in 2 cases (4.1%). Intra peritoneal effusion was objectified in 15 cases. The diagnosis of ovarian fibro-thecoma was raised based on U.S in 25 cases (51.02%) before surgery .MRI was performed in four cases. All patients underwent surgery. We performed laparotomy in 36 cases and laparoscopy in 11 cases. By laparotomy were performed a total hysterectomy with bilateral oophorectomy in most cases (26 patients). By laparoscopy we did lumpectomy in all cases. . The final pathologic examination revealed 19 fibromas, 14cystadénofibromas and 14 fibrothecomas. CONCLUSION: The paraclinical exploration of ovarian fibro-thecoma isbased, as all ovarian tumors, on ultrasound examination. The most typical features are images of solid tumors with regular contours, echogenic or mixed with the presence of streakedshadows.

Recurrent endometrial hyperplasia as a presentation of estrogen-secreting thecoma - case report and minireview of the literature.

Thecoma is a rare ovarian tumor, presenting usually in postmenopausal women as unilateral, benign, solid lesion. About 15% of affected patients develop endometrial hyperplasia (EH) and 20% are diagnosed with endometrial cancer. In this case report, we present 60-year-old women admitted because of recurrent spotting of 5 years duration, which started 1 year after menopause. In history, the patient underwent three times curettage procedures and once (1 year before admission) had estradiol levels typical for reproductive-age women. At admission, we found elevated serum levels of estradiol (222.5 pg/ml) and a small mass in the right ovary. The markers of germ cell tumors were negative. After the initial diagnosis, the patient was qualified for total abdominal hysterectomy with bilateral salpingo-oophorectomy. The histopathological examination and immunohistochemical staining confirmed the thecoma diagnosis. In follow-up examination after 8 weeks, we found decreased serum estradiol levels and relief of the symptoms. In conclusion, we want to underline that in cases of EH, especially in patients with a history of recurrences, the special attention should be paid for differential diagnosis. In such cases, the estrogen-secreting tumors should be excluded.

Value of diffusion-weighted imaging combined with conventional magnetic resonance imaging in the diagnosis of thecomas/fibrothecomas and their differential diagnosis with malignant pelvic solid tumors.

BACKGROUND: Our study aims to determine the value of diffusion-weighted imaging (DWI) combined with conventional magnetic resonance imaging (MRI) in the diagnosis of thecomas/fibrothecomas and their differential diagnosis with malignant pelvic solid tumors. METHODS: In total, 36 thecomas/fibrothecomas and 40 malignant pelvic solid tumors were included in our study. All patients underwent 1.5 T conventional MRI and DWI examinations except one patient with a fibrothecoma in whom DWI examination was not performed. The clinical features and characteristics of conventional MRI and DWI of these two groups were analyzed. Apparent diffusion coefficient (ADC) values were measured and compared between groups. Univariate analysis, multivariate logistic regression analysis, and the receiver operating characteristic curve were used for statistical analysis. RESULTS: All the thecomas/fibrothecomas showed isointensity on T1 weighted imaging (T1WI) and 77.8% (28/36) lesions showed hypo- to isointensity on T2 weighted imaging (T2WI). After administration of contrast medium, 94.4% (34/36) tumors appeared as minor to mild enhancement. On DWI, they showed a diversity of low to very high signal intensity. All malignant pelvic masses manifested as hyperintensity on T2WI and 87.5% (35/40) tumors showed very high signal (grade 3) on DWI. Higher area under the curve (AUC) and specificity could be achieved by using the lowest ADC value than the mean ADC value. Multivariate logistic regression analysis showed that shape, signal intensity on T2WI, capsule, and the lowest ADC value were the important indicators in discriminating thecomas/fibrothecomas from malignant pelvic solid tumors. CONCLUSIONS: The combination of DWI and conventional MRI is of great value in the diagnosis of thecomas/fibrothecomas and their differential diagnosis with malignant pelvic solid tumors.

Accuracy of intra-operative frozen section and its role in the diagnostic evaluation of ovarian tumors.

UNLABELLED: Summary OBJECTIVE: This retrospective study was undertaken to evaluate the accuracy and role of intra-operative frozen section in the diagnosis of ovarian tumors. MATERIALS AND METHODS: Retrospective study of 804 ovarian frozen section results between June 2010 and June 2014 was examined to determine the accuracy of frozen section diagnosis. The intra-operative frozen section diagnosis was compared with the permanent (paraffin) section and the overall accuracy, sensitivity, specificity, and positive and negative predictive values of the frozen section were studied. RESULTS: The overall accuracy to determine the status of malignancy was 92.6%. There were 38 (7.4%) false negative and no false positive frozen section diagnoses.The sensitivity, specificity, and positive predictive and negative predictive values for benign ovarian tumors were 100.0%, 97.0%, 91.3%, and 100.0%, respectively; for borderline tumors they were 64.3%, 97.0%, 91.5%, and 94.0%, respectively, and for malignant tumors they were 90.0%, 100.0%, 100.0%, and 85.5%, respectively. CONCLUSION: This study concluded that frozen section appears to be an adequate technique for the histopathological diagnosis of ovarian tumors, with some limitations observed among borderline and mucinous tumors.

Magnetic resonance imaging features of ovarian fibroma, fibrothecoma, and thecoma.

OBJECTIVE: To retrospectively evaluate the conventional and functional (diffusion- or perfusion-weighted) magnetic resonance (MR) imaging features of ovarian fibroma, fibrothecoma, and thecoma. MATERIALS AND METHODS: Histologically proven ovarian fibromas (n = 19), fibrothecomas (n = 7), and thecomas (n = 2) were reviewed (26 patients). The morphologic and signal intensity (SI) characteristics on conventional MR imaging (n = 28, all cases) were analyzed. The b 1000 signal intensity on diffusion-weighted image (DWI) (n = 22) and the time-to-signal intensity curve on perfusion-weighted image (PWI) (n = 7) were also analyzed. The presence and shape of the ipsilateral ovarian tissue surrounding the lesions were evaluated on T2-weighted image. RESULTS: Twenty-two cases (79%) were predominantly solid tumor. Majority of the detected lesions exhibited the characteristic homogeneous low SI on T1- (24/28, 86%) and T2- (19/28, 68%) weighted image. Conversely, a number of lesions exhibited high SI (9/28, 32%) on T2-weighted image. Most lesions presented with a detectable ipsilateral ovary on T2-weighted image (24/28, 86%). Tumors larger than 6 cm more likely showed atypical morphology (mixed solid and cystic, cystic), atypical SI (high on T1- and T2-weighted image), and large amount ascites. Larger tumor group (>6 cm) was more likely diagnosed as fibrothecoma or thecoma than fibroma by pathology. On DWI, 16 lesions showed low b 1000 signal intensity (16/22, 73%). On PWI, all lesions showed curve type 1 or 2 (7/7, 100%), which tends to characterize benign lesions. All (16/16, 100%) pre-menopausal women had a detectable ipsilateral ovary, and six (60%) out of 10 post-menopausal women had a detectable ipsilateral ovary (p < 0.05). CONCLUSIONS: Combining conventional morphologic and signal intensity characteristics with the findings from DWI or PWI might help differentiate ovarian fibroma, fibrothecoma, and thecoma from ovarian malignancy, although further prospective larger scale study using DWI and PWI is needed.

The detailed histopathological characteristics of ovarian fibroma compared with thecoma, granulosa cell tumor, and sclerosing stromal tumor.

BACKGROUND: Ovarian fibromas are benign tumors that can present peculiar morphological features not studied sufficiently. MATERIAL AND METHODS: In this retrospective study, 75 consecutive cases of ovarian fibroma were morphologically compared with 46 thecomas, 16 granulosa cell tumors, and 5 sclerosing stroma tumors for the following factors: the growth pattern as diffuse or nodular, the presence of hyaline plaques, necrosis, keloid-like sclerosis, calcifications, cystic degeneration, fibrous or edematous stroma, prominent vascularity, lutein cells, cellularity, scant or abundant cytoplasm, prominent cell membranes, nuclear grooves, atypia, and mitotic activity. RESULTS: The tumors differed significantly in terms of hyaline plaques presence, nuclear grooves, growth pattern, stroma type, tumor cellularity, cytoplasm, prominence of cell membranes, atypia, mitotic activity, and prominent vascularity. CONCLUSION: Ovarian fibromas can present some maybe unexpected features rather frequently, such as cystic degeneration, hyaline plaques, prominent vascularity, increased cellularity, and some mitotic activity, thus their presence should not always prompt to an alternative diagnosis.

Intraoperative frozen section evaluation of ovarian sex cord-stromal tumours and their mimics: a study of 121 cases with emphasis on potential diagnostic pitfalls.

Ovarian sex cord-stromal tumours (SCSTs) present diagnostic difficulties during frozen section (FS) consultations due to their diverse morphology. This study aimed to evaluate the accuracy of FS evaluation of SCSTs in our institution, as well as to examine the reasons leading to incorrect FS diagnosis. Cases mimicking SCSTs and diagnosed as such during FS were also highlighted. We analysed 121 ovarian SCST cases and their mimics which underwent FS consultations over a 10-year period, to evaluate FS accuracy, reasons for deferrals and discrepancies. FS diagnoses were concordant, deferred and discrepant compared to the final diagnosis in 50 (41.3%), 39 (32.2%) and 32 (26.5%) cases, respectively. Major discrepancies (9/121, 7.4%) were mostly related to the diagnosis of adult granulosa cell tumour (AGCT). A fibromatous AGCT was misinterpreted as fibroma on FS, while a cystic AGCT was called a benign cyst. Conversely, a mesonephric-like adenocarcinoma, a sertoliform endometrioid carcinoma and a thecoma were misinterpreted as AGCT on FS. Another discrepant case was a Krukenberg tumour with prominent fibromatous stroma in which malignant signet ring cells were overlooked and misinterpreted as fibroma. Minor discrepancies were primarily associated with fibroma (21/23, 91.3%), wherein minor but potentially impactful details such as cellular fibroma and mitotically active cellular fibroma were missed due to sampling issues and misinterpretation as leiomyoma. FS evaluation for ovarian SCSTs demonstrated an overall accuracy of 78.5%, 81.0% and 81.8% for benign, uncertain/low malignant potential and malignant categories, respectively. There was no FS-related adverse clinical impact in all cases with available follow-up information (120/121 cases). Intraoperative FS evaluation of ovarian SCSTs is challenging. A small number of cases were misinterpreted, with AGCTs being the primary group where errors occur. Awareness of common diagnostic pitfalls and difficulties, alongside application of a stepwise approach, including (1) obtaining comprehensive clinical information, (2) thorough macroscopic examination and directed sampling, (3) meticulous microscopic examination with consideration of pitfalls and mimics, (4) effective communication with surgeons in difficult cases, and (5) consultation of subspecialty colleagues in challenging cases, will enhance pathologists' reporting accuracy and management of such cases in the future.

Ovarian fibromas and fibrothecomas: sonographic correlation with computed tomography and magnetic resonance imaging: a 5-year single-institution experience.

OBJECTIVES: To evaluate imaging characteristics of ovarian fibromas and fibrothecomas and to identify select clinical markers and imaging features to help in their diagnosis. METHODS: Over a 5-year period, 18 of 29 women with histologically proven fibromas or fibrothecomas underwent sonography, computed tomography (CT), or magnetic resonance imaging (MRI). On review of the images, tumor size, solid component characteristics, and cystic components were evaluated. Age, cancer antigen 125 (CA-125), and Meig syndrome were assessed. RESULTS: Eleven fibrothecomas and 7 fibromas were evaluated. Sonography was performed for 15 tumors, CT for 9, and MRI for 6. Mean age was 52.6 (range, 13-82) years. Mean tumor size was 8.8 (range, 2-18) cm. Seventy-two percent of the tumors were solid, and 28% had cystic components. On sonography, the solid components were isoechoic or hypoechoic compared to the uterus. On CT with contrast, 2 of 8 lesions (25%) showed enhancement. On T1-weighted MRI, 5 lesions (83%) showed an isointense signal, and 1 (17%) showed a hyperintense signal compared to the myometrium. On T2-weighted MRI, 4 of 6 lesions (67%) were hypointense; 1 (16.5%) was isointense; and 1 (16.5%) was hyperintense. Elevated CA-125 was present in 5 of 29 patients (28%). One had Meig syndrome. CONCLUSIONS: For a cystic adnexal mass where the primary consideration is commonly an epithelial tumor, the possibility of a cystic stromal tumor should also be considered. Unlike previous studies reporting both T1 and T2 hypointensity, fibrothecomas and fibromas can also show T1 and T2 isointensity and, exceptionally, hyperintensity. Vascularity, shown by Doppler flow and MRI and CT enhancement, is a characteristic of some fibromas and fibrothecomas. Although CA-125 is elevated in some patients, a true correlation is difficult to assess. Meig syndrome is infrequent.

Clinical characteristics and surgical management options for ovarian fibroma/fibrothecoma: a study of 97 cases.

AIMS: The aims of this study were to analyze the clinical characteristics, diagnostic features, and operative outcomes of patients with ovarian fibroma/fibrothecoma and to discuss appropriate management options. METHODS: We performed a retrospective comparative analysis of 97 patients with ovarian fibroma/fibrothecoma who underwent laparoscopic and laparotomic procedures, including tumorectomy between January 2008 and December 2011. RESULTS: The mean patient age was 42.5 ± 11.4 years. Seventy-three patients (75.3%) were premenopausal women. A preoperative diagnosis of benign ovarian tumor or fibroma was made in only 49 cases (50.5%). We found that 42.2% of cases were misdiagnosed as uterine myomas (n = 41) and 6.2% were misdiagnosed as malignant ovarian tumors (n = 6). The presence of ascites was associated with larger tumor size (p < 0.05) but not higher CA125 levels (p = 0.159). Twenty-nine patients (29.9%) underwent laparotomy, and 68 (70.1%), laparoscopic surgery. Laparoscopic surgery facilitated shorter operation times than laparotomy (p < 0.05). Tumorectomy was performed in 40 patients (43.5%), 36 (90%) of whom underwent laparoscopy with operative outcomes comparable to those of patients who underwent laparotomy. CONCLUSIONS: Ovarian fibromas/fibrothecomas are often misdiagnosed as uterine myomas and malignant ovarian tumors. Laparoscopic surgery including tumorectomy may be an effective surgical approach in patients with ovarian fibromas/fibrothecomas.

Ovarian fibroma/fibrothecoma: retrospective cohort study shows limited value of risk of malignancy index score.

BACKGROUND: Ovarian fibromas/fibrothecomas are uncommon benign tumours of ovary. Due to their solid structure, these benign tumours are sometimes confused with malignant tumours during clinical evaluation. AIMS: To determine the clinico-pathological characteristics of ovarian fibroma/fibrothecoma and analyse the efficiency of risk of malignancy index (RMI) scoring system to distinguish malignancy among these tumours. METHODS: Between November 2001 and February 2012, women with a pathological diagnosis of ovarian fibroma/fibrothecoma were identified. Depending on the menopausal status, serum CA-125 level and ultrasonographic findings, RMI scores were calculated for each of the patients. RESULTS: During the study period, 43 ovarian fibroma/fibrothecoma (4.7%) were detected among 912 adnexal masses operated. The mean age of the women was 52.2 (range, 21-80 years). Upon calculating RMI scores, 33 women (76.7%) were classified as low risk and 10 women (23.3%) as high risk for malignancy. Sensitivity, specificity, positive predictive value and negative predictive value of the RMI scoring for identification of malignant lesions preoperatively were found as 0%, 76%, 0% and 97%, respectively. Final pathological diagnosis was ovarian fibroma in 13 (30%) women, fibrothecoma in 29 (67%) and fibrosarcoma in one woman (2%). CONCLUSION: There are no specific markers for accurate preoperative diagnosis of ovarian fibroma/fibrothecoma. Moreover, according to our results, RMI scoring system does not aid clinicians in this issue either, with a high false-positive rate and very low sensitivity. Further studies with higher number of cases are needed to state clearly the role of RMI scores in preoperative discrimination of malignancy.

Pathological discrimination between luteinized thecoma associated with sclerosing peritonitis and thecoma.

BACKGROUND: Similarities between luteinized thecoma associated with sclerosing peritonitis (LTSP) and thecoma, cause difficulty in clinical differential diagnoses. To improve the situation, we selected 10 specified molecular pathological markers that are frequently used in clinical pathology of ovarian sex cord-stromal tumors to determine whether they exert a discriminatory effect. METHODS: Applying immunohistochemistry, we analyzed the expression of alpha-1,6-mannosylglycoprotein 6-beta-n-acetylglucosaminyltransferase B (MGAT5B), nuclear receptor coactivator 3 (NCOA3), proliferation marker protein Ki-67 (MKI67), estrogen receptor, progesterone receptor, Vimentin, receptor tyrosine-protein kinase erbB-2, Catenin beta-1 (ß-Catenin), CD99 antigen (CD99) and Wilms tumor protein (WT1) in 102 cases of diseases containing 11 LTSP and 91 thecoma. Whole-exome sequencing and fluorescence in situ hybridization were used to examine the MGAT5B-NCOA3 fusion gene in LTSP. Statistical analysis was performed using t test, one-way analysis of variance test, and post hoc test. RESULTS: Six significant markers were verified for the discrimination between LTSP and thecoma, containing 4 upregulating indicators MGAT5B, NCOA3, MKI67, ß-Catenin, and 2 downregulating markers CD99 and WT1 in luteinized cells. In addition, the MGAT5B-NCOA3 fusion gene was identified in LTSP for the first time with significantly rich expression compared to thecoma. CONCLUSIONS: We verified 6 significant molecular pathological markers containing MGAT5B, NCOA3, MKI67, ß-Catenin, CD99, and WT1 and identified MGAT5B-NCOA3 fusion gene in LTSP; this work will help clinicians to discriminate between medical conditions and treat patients accurately.

Incidental FOXL2 mutated adult granulosa cell tumour of the ovary with thecoma-like foci.

We report on the incidental finding of a FOXL2 mutated adult granulosa cell tumour of the ovary with thecoma-like foci, a rare entity recently described by Jennifer N. Stall and Robert H. Young in a series of sixteen cases in 2019, displaying features differing from conventional adult granulosa cell tumour. Our aim is to specify the morphologic and molecular particularities of this presumably underrecognized finding, with a short presentation of the typical clinical context. Awareness of this rare and challenging neoplasm with indeterminate clinical course is crucial in routine diagnostics.

Preliminary observations and diagnostic value of lipid peak in ovarian thecomas/fibrothecomas using in vivo proton MR spectroscopy at 3T.

PURPOSE: To retrospectively evaluate the significance of lipid peak in in vivo proton magnetic resonance (MR) spectroscopy for the diagnosis of ovarian thecomas/fibrothecomas in patients with solid gynecologic tumors exhibiting totally or partially low signal intensity on T2-weighted images. MATERIALS AND METHODS: MR spectroscopy was performed in patients with pathologically diagnosed gynecologic tumors at 3T MRI. Single-voxel MR spectroscopy data were collected from a single square volume of interest that encompassed the gynecologic masses. The lipid concentration level was classified into three classes (high; low; none). RESULTS: A total of 20 gynecologic tumors in 20 patients were evaluated in this study. High lipid peak was observed in all seven thecomas/fibrothecomas, whereas low lipid peak was observed in only one fibroma in 13 nonthecomatous tumors (six benign ovarian tumors and seven subserosal uterine leiomyomas). The presence of lipid peak for the diagnosis of thecomas/fibrothecomas had a sensitivity of 100%, specificity of 92%, positive predictive value of 88%, and negative predictive value of 100%. CONCLUSION: The high lipid peak reflecting abundant intracellular lipid contents is considered a specific metabolite concentration for thecomas/fibrothecomas. Demonstration of high lipid peak may contribute to the diagnosis of thecomas/fibrothecomas in distinguishing from other benign ovarian fibrous tumors or subserosal uterine leiomyomas.

MRI features of ovarian fibroma and fibrothecoma with histopathologic correlation.

OBJECTIVE: The purpose of this article is to evaluate MRI features of ovarian fibroma and fibrothecoma with histopathologic correlation. MATERIALS AND METHODS: In this retrospective study, preoperative MRI examinations of 35 women (mean age, 49 years; range, 24-86 years) with pathologically proven ovarian fibroma (n = 25) or fibrothecoma (n = 10) were reviewed by two radiologists in consensus. MRI features, including visibility of ovaries, presence of capsule, degeneration, T1 and T2 signal, and enhancement pattern, were recorded and correlated with histopathologic features. After administration of gadopentetate dimeglumine, the maximum percentages of enhancement of fibroma or fibrothecoma, myometrium, and, if present, uterine fibroids (11/35 patients) were compared. RESULTS: All fibromas and fibrothecomas appeared well defined, with a mean size of 6.36 × 4.81 cm. Ipsilateral and contralateral ovaries were each seen in 89% (31/35) of patients. Most fibromas and fibrothecomas were isointense to hypointense compared with myometrium on T1-weighted (91% [32/35]) and T2-weighted (77% [27/35]) images. Capsule was noted in 63% (22/35) and degenerative changes were noted in 66% (23/35) of patients. Fibromas and fibrothecomas larger than 6 cm more likely showed capsule (p < 0.0001, Fisher exact probability test), degenerative changes (p = 0.003), peripheral subcapsular cystic areas (p < 0.0001), heterogeneous T2 signal (p = 0.001), and heterogeneous enhancement (p = 0.005). At least four of the above five characteristics were present in 93% (14/15) of fibromas and fibrothecomas larger than 6 cm (p < 0.0001). The maximum percentage of enhancement for fibromas and fibrothecomas (63%) was significantly lower than those for myometrium (131%; p < 0.0001) and fibroids (103%; p < 0.0001), without a statistically significant difference between the maximum percentage enhancement of myometrium and fibroids. A maximum percentage of enhancement less than 75% yielded 92% positive predictive value in differentiating fibromas and fibrothecomas from fibroids. Fibrothecomas had a higher maximum percentage of enhancement than did fibromas (p = 0.01). CONCLUSION: MRI features of ovarian fibromas and fibrothecomas depend on size, with capsule and degenerative changes common with fibromas and fibrothecomas larger than 6 cm. Fibromas and fibrothecomas enhance less than myometrium and fibroids do, and less than 75% maximum percentage enhancement can help in differentiating fibromas and fibrothecomas from fibroids.