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.

MRI combined with clinical features to differentiate ovarian thecoma-fibroma with cystic degeneration from ovary adenofibroma.

OBJECTIVE: To explore the value of magnetic resonance imaging (MRI) and clinical features in identifying ovarian thecoma-fibroma (OTF) with cystic degeneration and ovary adenofibroma (OAF). METHODS: A total of 40 patients with OTF (OTF group) and 28 patients with OAF (OAF group) were included in this retrospective study. Univariable and multivariable analyses were performed on clinical features and MRI between the two groups, and the receiver operating characteristic (ROC) curve was plotted to estimate the optimal threshold and predictive performance. RESULTS: The OTF group had smaller cyst degeneration degree (P < .001), fewer black sponge sign (20% vs. 53.6%, P = .004), lower minimum apparent diffusion coefficient value (ADCmin) (0.986 (0.152) vs. 1.255 (0.370), P < .001), higher age (57.4 ± 14.2 vs. 44.1 ± 15.9, P = .001) and more postmenopausal women (72.5% vs. 28.6%, P < .001) than OAF. The area under the curve of MRI, clinical features and MRI combined with clinical features was 0.870, 0.841, and 0.954, respectively, and MRI combined with clinical features was significantly higher than the other two (P < .05). CONCLUSION: The cyst degeneration degree, black sponge sign, ADCmin, age and menopause were independent factors in identifying OTF with cystic degeneration and OAF. The combination of MRI and clinical features has a good effect on the identification of the two. ADVANCES IN KNOWLEDGE: This is the first time to distinguish OTF with cystic degeneration from OAF by combining MRI and clinical features. It shows the diagnostic performance of MRI, clinical features, and combination of the two. This will facilitate the discriminability and awareness of these two diseases among radiologists and gynaecologists.

Coexisting ovarian serous cystadenoma with fibroma: A very unusual combination.

Surface epithelial neoplasms are the most common ovarian tumors, constituting around 60% of all ovarian malignancies. They are classified as benign, borderline, and malignant. Ovarian cystadenomas are common benign epithelial neoplasms which carry an excellent prognosis. Ovarian thecoma-fibroma groups are uncommon sex cord-stromal neoplasms, constituting 1.0%-4.0% of all ovarian tumors. Most of them are benign and often found in postmenopausal patients. Combination tumors in the ovary are known. The most common combination is mucinous cystadenoma which occurs in association with Brenner tumor, mature cystic teratoma, Sertoli-Leydig cell tumor, or even a serous cystadenoma. A combination of surface epithelial and thecoma-fibroma group is very rarely encountered. A case of one such combination of serous cystadenoma and fibroma of the ovary is being presented here in a postmenopausal woman.

A case report of two pelviscopic resections of fibrothecomas originating from the left ovary with recurrence after ten years.

RATIONALE: Fibrothecomas are benign ovarians tumors. These are solid sex-cord-stromal tumors, accounting for 1% to 4.7% of all ovarian neoplasms. Their recurrence rate is known to be only 2% following ovarian sparing local mass excision. We report an uncommon case of 2 pelviscopic resections of fibrothecomas originating from the left ovary with recurrence after 10 years in a 34-year-old woman. PATIENT CONCERNS: A 34-year-old married woman was diagnosed with 41 mm sized left ovarian recurrent fibrothecoma. We performed mass excision pelviscopically the first time 10 years ago. She gave birth to her second baby at 7 years after the first surgery. Ten years after the first surgery, fibrothecoma recurred on the same ovary with size larger than before. DIAGNOSES: At the time of its first occurrence 10 years ago, the ultrasound scan revealed a 34 × 23 mm-sized solid hypoechoic mass with well-demarcated margins and minimal Doppler flows. Ultrasound findings at the time of recurrence 10 years later showed the same findings, with its size increased to 41 × 40 mm. Final pathologic findings showed left ovarian fibrothecoma. INTERVENTIONS: After her admission to the hospital, we performed pelviscopic removal of left ovarian fibroma. Microscopic examination revealed predominantly bland spindle cells with collagenous stroma, showing fascicular and storiform growth. OUTCOMES: Surgeries were successful. The patient had been followed-up regularly for 3 years after last surgery. She did not experience any complications. She remained disease-free. LESSONS: Repetitive local mass excision appears to be an effective surgical option in women of reproductive age. Although there is a sufficient possibility of recurrence several years to decades after only mass excision, mass excision is more appropriate than total oophorectomy in women of childbearing age. Pelviscopic surgery is recommended.

TFE3 nuclear expression as a novel biomarker of ovarian sclerosing stromal tumors and associated with its histological morphology.

Sclerosing stromal tumors of the ovary are benign and tend to occur in youthful women with lobular structures at low frequencies. Three types of cells, including luteinized cells, short spindle myoid cells, and intermediate cells, are found in the lobules which abundant in the blood vessels. Currently, immunohistochemistry is used to detect normal follicles, sclerosing stromal tumors, granulosa cell tumors, and fibromas/thecomas. Our research results showed that transcription factor enhancer 3 (TFE3) was moderate to strong positive in the theca interna layer of normal follicles. TFE3 was expressed in seven out of eight sclerosing stromal tumors, mainly in luteinized cells. It did not express in 20 granulosa cell tumors. Of the nine fibromas/thecomas, TFE3 was weakly staining in 2 cases and negative in the remaining 7 cases. The expression of TFE3 was also weak in only one microcystic stromal tumor. 8 cases of sclerosing stromal tumors were analyzed by FISH using a TFE3 separation probe, and the results were negative. In short, as a nuclear transcription protein, TFE3 specifically expressed in sclerosing stromal tumors and could serve as a new marker for the diagnosis and differential diagnosis of sclerosing stromal tumors. Moreover, we speculate that TFE3 will promotes the formation of the vascular plexus after entry into the nucleus, which can further explain why sclerosing stromal tumors are different from other ovary sex-cord stromal tumors.

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.

Sclerosis in Sex Cord-Stromal Tumors Other Than the Sclerosing Stromal Tumor: A Report of 70 Cases.

Sclerosis is well-known in sclerosing stromal tumors (SSTs), as its name indicates, but has not been evaluated in other ovarian sex cord-stromal tumors (SCSTs). Its presence in other SCSTs has sporadically caused diagnostic problems in cases we have seen, and this prompted us to review SCSTs with appreciable sclerosis; tumors containing at least 20% sclerosis were included. Seventy cases were identified: 20 thecomas, 20 juvenile granulosa cell tumors (JGCTs), 8 adult granulosa cell tumors (AGCTs), 5 sex cord tumors with annular tubules, 6 retiform Sertoli-Leydig cell tumors (SLCTs; all of the intermediate differentiation), 4 nonretiform SLCTs (3 well-differentiated, 1 of intermediate differentiation with heterologous elements), 4 Sertoli cell tumors, and 3 microcystic stromal tumors (MSTs). Paucicellular sclerotic zones comprised 20% to 95% of the tumors and when conspicuous often obscured diagnostic features. Thirty-one tumors (10 thecomas, 19 JGCTs, 1 AGCT, and 1 MST) showed sclerotic zones focally enveloping nodules of tumor cells, imparting a pseudolobular appearance, and sclerosis often occurred within lobules as well. Ten of these (5 thecomas and 5 JGCTs) also had prominent staghorn blood vessels, generating a low-power appearance focally similar to SST. In 17 tumors, the sclerosis resulted in "compression" of the tumor cells into cords and/or solid tubules. Correct diagnosis in these cases is dependent on careful examination of the cellular zones of the neoplasms, but awareness of the extent of sclerosis that may be seen in diverse SCSTs may be crucial in suggesting the correct diagnosis particularly when the material is limited as in the intraoperative setting. Our findings highlight for the first time the occurrence and character of sclerosis in sex cord tumors other than SSTs and fibromas. Sclerosis is seen in descending proportion of the tumor types as follows: retiform SLCTs, thecomas, MSTs, JGCTs, sex cord tumors with annular tubules, Sertoli cell tumors, AGCTs, and nonretiform SLCTs. Its character can vary somewhat, having particular features in the sex cord tumor with annular tubules (hyaline material within tubules often coalescing and extending beyond the nests to form confluent aggregates) and retiform SLCTs (common in papillary cores).

Fibrothecoma of broad ligament with minor sex cord elements: Case report and brief literature review.

RATIONALE: Sex cord-stromal tumors are always found in ovary, but the occurrence of this kind of tumor at extraovarian locations is extremely rare. Up to now, the case concerning fibrothecoma of broad ligament with minor sex cord elements has not been reported, and it is extremely challenging to diagnose before surgery. In this case report, we summarized pathogenesis, clinical features, laboratory finding, imaging studies, pathology, and therapeutic schedule of this tumor, with the aim of raising awareness and attention to this type of disease. PATIENT CONCERNS: A 45-year-old Chinese woman was referred to our department with intermittent lower abdominal pain for about 6 years. On examination, both ultrasonography and computed tomography revealed she had a right adnexal mass. DIAGNOSIS: Based on the results of histology and immunohistochemistry, the final diagnosis was confirmed as fibrothecoma of broad ligament with minor sex cord elements. INTERVENTIONS: This patient underwent laparoscopic unilateral salpingo-oophorectomy with excision of the neoplasm. OUTCOMES: Eleven days post-treatment, the patient complained that the symptoms of abdominal pain was disappeared. There is no evidence of disease recurrence 5 years after laparoscopic surgery according to the consequences of radiologic examination. CONCLUSION: The natural history of this kind of tumor is uncertain. Although main treatment of this neoplasm might be surgical resection and good prognosis can be achieved, we believe that long-time follow-up is extremely important in all patients diagnosed as fibrothecoma of broad ligament with minor sex cord. Laparoscopic unilateral salpingo-oophorectomy with excision of the tumor should be recommended to these patients.

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.

Diagnostic utility of a conventional MRI-based analysis and texture analysis for discriminating between ovarian thecoma-fibroma groups and ovarian granulosa cell tumors.

OBJECTIVE: To evaluate the diagnostic utility of conventional magnetic resonance imaging (MRI)-based characteristics and a texture analysis (TA) for discriminating between ovarian thecoma-fibroma groups (OTFGs) and ovarian granulosa cell tumors (OGCTs). METHODS: This retrospective multicenter study enrolled 52 patients with 32 OGCTs and 21 OTFGs, which were dissected and pathologically diagnosed between January 2008 and December 2019. MRI-based features (MBFs) and texture features (TFs) were evaluated and compared between OTFGs and OGCTs. A least absolute shrinkage and selection operator (LASSO) regression analysis was performed to select features and construct the discriminating model. ROC analyses were conducted on MBFs, TFs, and their combination to discriminate between the two diseases. RESULTS: We selected 3 features with the highest absolute value of the LASSO regression coefficient for each model: the apparent diffusion coefficient (ADC), peripheral cystic area, and contrast enhancement in the venous phase (VCE) for the MRI-based model; the 10th percentile, difference variance, and maximal correlation coefficient for the TA-based model; and ADC, VCE, and the difference variance for the combination model. The areas under the curves of the constructed models were 0.938, 0.817, and 0.941, respectively. The diagnostic performance of the MRI-based and combination models was similar (p = 0.38), but significantly better than that of the TA-based model (p < 0.05). CONCLUSIONS: The conventional MRI-based analysis has potential as a method to differentiate OTFGs from OGCTs. TA did not appear to be of any additional benefit. Further studies are needed on the use of these methods for a preoperative differential diagnosis of these two diseases.

CT review of ovarian fibrothecoma.

OBJECTIVE: The aim of this study was to investigate the CT imaging characteristics of ovarian fibrothecoma which may aid in the differentiation from early stage epithelial tumours. METHODS: Comparison of 36 patients (41 lesions) with pathologically proven ovarian fibrothecoma tumours and 36 (52 lesions) serous papillary carcinomas (SPCs) lesions. We noted their laterality, size, density, calcifications, Hounsfield units (HUs) and introduced a novel HU comparison technique with the psoas muscle or the uterus. Patients' clinical findings such as ascites, pleural effusion, carbohydrate antigen-125 levels, and lymphadenopathy findings were also included. RESULTS: Average age was 67.8 and 66 across the fibrothecoma and SPC cohort respectively. Fibrothecoma tumours had diameters ranging from 24 to 207 mm (Median: 94 mm). 80.6% of the fibrothecoma cohort had ascites which was comparable to the 72.2% in the SPC cohort. 70.7% of fibrothecoma tumour favour a purely to predominantly solid structural configuration (p < 0.001). The average HU value for the fibrothecoma solid component was 44 ± 11.7 contrasting the SPC HU value of 66.8 ± 15. The psoas:tumour mass ratio demonstrated a median of 0.7, whereas SPCs shows a median of 1.1 (p < 0.001). CONCLUSION: Suspicion of ovarian fibrothecoma should be considered through interrogation of their structural density configuration, low psoas to mass HU ratio and a presence of ascites. ADVANCES IN KNOWLEDGE: CT imaging can be a useful tool in diagnosing fibrothecoma tumours and subsequently reducing oncogynaecological tertiary centre referrals, financial burden and patient operative morbidity and mortality.

Synchronous uterine serous carcinoma and ovarian sex cord stromal tumor (thecoma)-A rare first case report.

Synchronous endometrial and ovarian carcinoma is a rare instance and it accounts for 50 to 70% of all synchronous female genital tract tumors. However, it is very rare to find synchronous endometrial carcinoma and ovarian sex cord-stromal tumor (thecoma). The present case is a 75-year-old woman with a complaint of post-menopausal vaginal bleeding. Radiologically, the magnetic resonance imaging (MRI) pelvis revealed altered signal intensity mass in the uterus. Frozen section and routine histopathological examination were done on radical hysterectomy. Microscopically, serous carcinoma involving uterine corpus and left Fallopian tube was identified along with the unusual finding of contralateral ovarian sex cord-stromal tumor (thecoma), which was confirmed on immunohistochemical examination. It is a very rare association and is first reported in the present study after a thorough search of the published literature. Their relationship based on a high level of estrogen produced by the hyperactive ovary is controversial as serous carcinomas are less hormone-dependent.

Brenner tumors.

Brenner tumors are rare ovarian neoplasms composed of ovarian transition cells surrounded by dense fibrous tissue. Most of them are small tumors (<2 cm), detected incidentally in asymptomatic women. Its predominantly fibrous content results in relatively low signal on T2 weighted images, establishing differential diagnosis with ovarian fibroma and thecoma. Their imaging features are very similar, the differentiation is based on secondary characteristics, such as signs or symptoms of estrogen excess and the presence of a second ovarian neoplasm, which has been reported in up to 30% of patients with Brenner tumor. Although originally thought to be universally benign, there have been scattered reports in the past decades of borderline and malignant forms of Brenner tumors.

Pelvic effusion in patients with ovarian thecoma-fibroma associated with the tumor size and plaste CA-125 level: A retrospective magnetic resonance imaging study.

PURPOSE: To survey the occurrence rate of ascites in patients with thecoma-fibroma and its potential correlation with tumor MR imaging characteristics. METHODS: A total of 40 patients with surgically proven thecoma-fibroma were enrolled in this retrospective study. We determined the tumor size, the components (solid or cystic) and their signal intensity features. Second, we identified ascites according to the fat-suppressed sagittal T2-weighted imaging sequence and divided all cases into two groups (with or without ascites). Furthermore, we explored the correlations of ascites with tumor size, tumor solidity, pathological types, patient's postmenopausal status and serum CA-125 levels by using the χ2 test. RESULTS: (1) Among the 40 cases, 15 tumors were fibromas, 15 thecomas, and 10 fibrothecomas. Nine patients (26.47%) had elevated CA-125 levels (>35.0 U/ml). (2) Thirty-one patients had ascites (77.50%), 29 of which had a small amount of ascites. Nine cases had no ascites (22.50%). (3) MRI showed a solid mass in 22 cases (55.0%), cystic mass in five cases (12.5%) and mixed solid-cystic mass in 13 cases (32.5%). The χ2 test revealed that the incidence of ascites was significantly correlated with tumor size, tumor solidity and serum CA-125 levels (P < 0.05), but not with menopause and pathological type (P > 0.05). CONCLUSION: Our data revealed that the incidence of ascites was 77.50% and was mainly correlated with tumor size and elevated CA-125 levels. These findings have potential value for improving the diagnosis and differential diagnosis of thecoma-fibroma.

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.

Ovarian thecoma: A very unusual cause of postmenopausal bleeding.

We describe a 67-year-old woman with postmenopausal bleeding having ceased estrogen plus progestogen therapy nine months before. Transvaginal ultrasonography showed endometrial thickening with normal ovarian appearance. Hormonal studies revealed high estradiol and inhibin B levels but normal androgens and adrenal hormones. Magnetic resonance image demonstrated a 13-mm left ovarian tumour. Hysterectomy and bilateral salpingo-oophorectomy were performed, and the pathological study revealed an 8 mm ovarian thecoma. This case illustrates a very unusual cause of postmenopausal bleeding. We suggest a study protocol and discuss the differential diagnosis of this case.

COMPLETE REMISSION OF MEDICALLY TREATED LUTEINIZED THECOMAS WITH SCLEROSING PERITONITIS.

We describe the first French patient treated for sclerosing peritonitis syndrome associated with lutheinized thecomas. At 42 years old, she had respiratory distress with increased abdominal volume. Physical examination revealed ascites, pleural effusions, and two mobile latero-uterine masses. Radiological examinations revealed bilateral ovarian masses of 10 cm. Bilateral adnexectomy was performed by laparotomy. Histological analysis concluded that there were benign luteinized thecomas. Until the 36th postoperative day, the general condition of the patient deteriorated to become critical. A second surgical procedure was attempted revealing sclerosing fibrosis preventing access to the peritoneal cavity. Subsequently, a medical treatment combining parenteral nutrition, high intravenous doses of corticosteroids, antiestrogens, colchicine and sandostatin was administered and effective allowing continuity recovery 15 months later. The clinical outcomes has been favorable at 24 month later.