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The British Journal of Radiology Feb 2022Brenner tumors are rare ovarian neoplasms composed of ovarian transition cells surrounded by dense fibrous tissue. Most of them are small tumors (<2 cm), detected...
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 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.
Topics: Brenner Tumor; Cystadenofibroma; Diagnosis, Differential; Female; Fibroma; Humans; Leiomyoma; Magnetic Resonance Imaging; Ovarian Neoplasms; Rare Diseases; Thecoma; Tomography, X-Ray Computed; Ultrasonography
PubMed: 34928171
DOI: 10.1259/bjr.20210687 -
Pathology Jan 2018Sex cord-stromal tumours of the ovary include many of the most morphologically intriguing ovarian neoplasms and albeit many of them are rare, they factor into the... (Review)
Review
Sex cord-stromal tumours of the ovary include many of the most morphologically intriguing ovarian neoplasms and albeit many of them are rare, they factor into the differential diagnosis more often than their frequency might suggest. The most common malignant form, the adult granulosa cell tumour, may grossly simulate various surface epithelial neoplasms. Microscopically, confusion with endometrioid carcinoma may occur because the cords and microfollicles of the granulosa cell tumour may be mimicked by endometrioid carcinoma and the latter may have pale nuclei with nuclear grooves. Thorough sampling generally resolves this differential and if not immunohistochemistry aids. Although the adult granulosa cell tumour typically has cells with scant cytoplasm in some cases the tumour cells are luteinised and others have cells with abundant pale cytoplasm. A reticulum stain may be of great aid in indicating whether cells of the type just noted are of granulosa or theca nature. Variations in the morphology of the juvenile variant of granulosa cell tumour that can be diagnostically challenging include those that have a macronodular pattern with scant follicular differentiation, those with marked sclerosis, and those that are unusually pleomorphic. The uncommon but histologically varied Sertoli-Leydig cell tumour is considered, emphasis being placed on the most recently described variant, the retiform pattern, with its potential to mimic surface epithelial neoplasms and even mixed mesodermal tumours. Considering the usual young age of the patient may be paramount in making this tumour come to the mind of the pathologist. The rare pure Sertoli cell tumour is briefly noted as is the sex cord tumour with annular tubules, well known because of its association in some cases with Peutz-Jeghers syndrome. Most do not have that association, however, but have their own interesting features including a greater than average risk, among sex cord stromal tumours, of nodal metastasis and progesterone production, and an occasional development from them of an otherwise typical Sertoli cell tumour. The stromal family includes the common fibroma which is challenging when it is cellular with some mitotic activity and the approach to such neoplasms is reviewed. Emphasis in the consideration of thecoma is placed on its typical cytological features and the overlap with what may be seen in some adult granulosa cell tumours. The review concludes with three fascinating pure stromal tumours all described within the last several decades: the sclerosing stromal tumour, the unusual luteinised thecoma associated with sclerosing peritonitis and the microcystic stromal tumour. The first is sometimes misdiagnosed when pure stromal neoplasms of other types are vascular and may have pseudolobules and it is essential that the pseudolobules of the sclerosing stromal tumour contain a haphazard admixture of fibroblasts and weakly luteinised cells. The remarkable tumours associated with peritonitis exhibit brisk mitotic activity but appear not to have a metastatic potential; they can cause significant problems because of the sclerosing peritonitis. The microcystic stromal tumour may mimic a steroid cell tumour or thecoma but unlike them is inhibin and calretinin negative, and stains for CD10 and β-catenin. It often shows bizarre nuclei atypia but limited mitotic activity and appears to be clinically benign on the basis of still limited experience.
Topics: Biomarkers, Tumor; Diagnosis, Differential; Female; Granulosa Cell Tumor; Humans; Immunohistochemistry; Ovarian Neoplasms; Ovary; Sex Cord-Gonadal Stromal Tumors
PubMed: 29132723
DOI: 10.1016/j.pathol.2017.09.007 -
Mayo Clinic Proceedings Aug 2019
Topics: Abdominal Pain; Biopsy, Needle; Female; Humans; Immunohistochemistry; Middle Aged; Ovarian Neoplasms; Ovariectomy; Postmenopause; Prognosis; Thecoma
PubMed: 31378246
DOI: 10.1016/j.mayocp.2019.04.019 -
International Journal of Surgical... Sep 2019
Topics: Female; Fibroma; Humans; Hysterectomy; Lutein; Middle Aged; Neoplasms, Complex and Mixed; Ovarian Neoplasms; Ovary; Salpingo-oophorectomy; Thecoma
PubMed: 30520326
DOI: 10.1177/1066896918817372 -
Medicine Jun 2023Similarities between luteinized thecoma associated with sclerosing peritonitis (LTSP) and thecoma, cause difficulty in clinical differential diagnoses. To improve the...
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 (β-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, β-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, β-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.
Topics: Female; Humans; Thecoma; beta Catenin; In Situ Hybridization, Fluorescence; Peritonitis; Ovarian Neoplasms; Peritoneal Fibrosis
PubMed: 37335673
DOI: 10.1097/MD.0000000000033911 -
The British Journal of Radiology Aug 2022The 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.
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 ( < 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 ( < 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.
Topics: Ascites; CA-125 Antigen; Female; Fibroma; Granulosa Cell Tumor; Humans; Neoplasms, Glandular and Epithelial; Ovarian Neoplasms; Retrospective Studies; Thecoma; Tomography, X-Ray Computed
PubMed: 35451310
DOI: 10.1259/bjr.20210790 -
Surgical Pathology Clinics Jun 2019Ovarian sex cord-stromal tumors are uncommon tumors and clinically differ from epithelial tumors. They occur across a wide age range and patients often present with... (Review)
Review
Ovarian sex cord-stromal tumors are uncommon tumors and clinically differ from epithelial tumors. They occur across a wide age range and patients often present with hormone-related symptoms. Most are associated with an indolent clinical course. Sex cord-stromal tumors are classified into 3 main categories: pure stromal tumors, pure sex cord tumors, and mixed sex cord-stromal tumors. The rarity, overlapping histomorphology and immunoprofile of various sex cord-stromal tumors often contributes to diagnostic difficulties. This article describes the various types of ovarian sex cord-stromal tumors and includes practical approaches to differential diagnoses and updates in classification.
Topics: Biomarkers, Tumor; Diagnosis, Differential; Female; Granulosa Cell Tumor; Humans; Leydig Cell Tumor; Ovarian Neoplasms; Sertoli Cell Tumor; Sex Cord-Gonadal Stromal Tumors; Thecoma
PubMed: 31097116
DOI: 10.1016/j.path.2019.02.005 -
Journal of Ovarian Research Nov 2016Ovarian thecoma-fibroma groups (OTFG) are uncommon sex cord-stromal neoplasms. The objective of the study was to demonstrate clinical and sonographic features of OTFG... (Comparative Study)
Comparative Study
BACKGROUND
Ovarian thecoma-fibroma groups (OTFG) are uncommon sex cord-stromal neoplasms. The objective of the study was to demonstrate clinical and sonographic features of OTFG and compare with surgical histopathology.
METHODS
A total of 61 patients with surgically proven OTFG were enrolled in this retrospective study to demonstrate its clinical and sonographic features and to compare with pathological findings. Gray scale and color Doppler sonography were performed presurgically with either transabdominal or transvaginal approach to image pelvic structures and lesions. The clinical findings and sonographic appearances were compared with the types of the OTFG tumors based on the histopathological diagnosis.
RESULTS
The mean patient age was 53.57 (range, 26-86) years. There were 63.93% (39/61) patients in postmenopausal and 63.93% (39/61) patients with no clinical symptoms. Ultrasound findings of OTFG revealed as solid tumors with a typical feature of well-demarcated hypoechoic masses in 70.49% (43/61), among which 74.41% (32/43) tumors were smaller than 5 cm in diameter. There were 17 mixed echogenic masses with calcification, hemorrhage, or cyst, among which 70.59% (12/17) lesions were larger than 5 cm in diameter. Acoustic attenuation of the tumor was presented in 44.26% (27/61) of the cases. Doppler flow signals within the tumors were found in 20 cases (32.79%), in which 80% (16/20) had minimal or moderate flow signals. Ascites was detected in 32.79% (20/61) of the cases, Megi's syndrome was found in 1 case. Final pathology revealed 41 (67.21%) thecoma-fibromas, 15 (24.59%) fibromas, 4 (6.56%) thecomas and 1 (1.64%) fibrosarcoma. There were 58 patients underwent cancer antigen 125 (CA125) test, and 20.69% (12/58) showed an elevated level. The diameter of tumors was found to be significantly correlated with CA125 level (p < 0.01) and the amount of ascites fluid (p < 0.05).
CONCLUSIONS
The typical sonographic features of OTFG include adnexal hypoechoic masses with clear border and acoustic attenuation as well as minimal Doppler flow signals. All the aforementioned features could make ultrasound imaging as a assistent tool improve the preoperative diagnostic accuracy.
Topics: Adult; Aged; Aged, 80 and over; CA-125 Antigen; Female; Fibroma; Humans; Membrane Proteins; Middle Aged; Ovarian Neoplasms; Postmenopause; Retrospective Studies; Sensitivity and Specificity; Thecoma; Ultrasonography, Doppler, Color
PubMed: 27876070
DOI: 10.1186/s13048-016-0291-2 -
Journal of Obstetrics and Gynaecology... Aug 2022Nearly 90% of all the hormone-producing ovarian tumours are sex cord-stromal tumours (SCSTs). The Ovarian fibroma is a hormonally inactive variant of SCST. It is...
BACKGROUND
Nearly 90% of all the hormone-producing ovarian tumours are sex cord-stromal tumours (SCSTs). The Ovarian fibroma is a hormonally inactive variant of SCST. It is composed of spindle, oval, round cells producing collagen and accounts for approximately 4% of all ovarian neoplasms. Amongst the other SCSTs, Inhibin B is an important tumour marker. It is a heterodimeric glycoprotein hormone that is secreted primarily by the granulosa cells of the developing follicles. High levels of Inhibin-B can hamper follicular recruitment, leading to amenorrhea in a reproductive age woman.
FINDING
In this case report, we describe a rare case of a reproductive age female presenting with secondary amenorrhea, having an Ovarian Fibroma, producing massive amounts of Inhibin B.
SIGNIFICANCE
Although some pathological variants of ovarian fibromas like cyst-adeno-fibroma and ovarian fibro-thecoma are known to secrete inhibin B, benign /pure ovarian fibromas rarely do so.
PubMed: 35923510
DOI: 10.1007/s13224-021-01601-y