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Background Sclerosing stromal tumors (SST) are rare ovarian neoplasms that often appear as solid unilateral tumors of the ovary with no specific clinical or radiological presentation. The definitive treatment is surgical removal. Case presentation Our article presents four cases of female patients with sclerosing stromal ovarian tumor with clinical characteristics mimicking malignant ovarian lesions. Interestingly, two of our cases had elevated levels of inhibin B. All patients were treated with surgery (oophorectomy) and had no disease recurrence. Conclusion Tumors’ macroscopic features are usually non-specific and often suggestive of possible malignancy, therefore diagnosis is always based on histopathological report.

Sclerosing stromal tumor of the ovary (SSTO) is a rare benign neoplasm of the ovary, accounting for about 6% of all sex cord stromal ovarian tumors. Only 17 cases of SSTO occurring during pregnancy have been reported to date. We report a case of SSTO occurring during pregnancy and review the existing literature. A 32-year-old pregnant woman was found to have a 10-cm solid mass in the right adnexa, and a large volume of ascites fluid was detected by ultrasound examination in the second trimester. The patient underwent abdominal puncture to relieve her symptoms in the second trimester, and a partial right oophorectomy and cesarean section were performed at 39 weeks of gestation. Final pathology confirmed the diagnosis of SSTO. Both the mother and baby were well at 6 months of postpartum follow-up. Clinical symptoms, and hormone and imaging examinations are all helpful in making a differential diagnosis of SSTO, but the unique histopathological and immunohistochemistry findings remain the main diagnostic features. Pregnant women with SSTO usually undergo enucleation or unilateral oophorectomy, which generally does not have adverse pregnancy outcomes.

Overin sklerozan stromal tümörü oldukça nadir görülen benign nonepitelyal bir tümördür. Hastalar genellikle pelvik ağrı, adet düzensizliği ve ele gelen kitle nedeniyle başvururlar. Bazen de hormonal olarak aktif olup androjen fazlalığı semptomlarına yol açabilmektedir. Tümörün radyolojik görüntüsü ve makroskopisindeki solid yapılar malign izlenim verebilmektedir. Kesin tanısı çoğunlukla postoperatif histolojik inceleme ile konulmaktadır. Biz bu olgu sunumunda oldukça nadir görülen sklerozan stromal tümörün klinik ve histopatolojik özelliklerini ve olgu yönetimini sunmayı amaçladık.

Sclerosing stromal tumor (SST) is a rare ovarian tumor arising from the sex cord-stromal cells that occurs mainly in young adults during the second and third decades of life and rarely in pediatric and adolescent populations. The objective of this study is to report three illustrative cases of SST in young girls who had undergone surgery at our clinic in or after 2009, and to perform a literature review of this rare ovarian tumor. A retrospective chart review of female patients aged <18 years with a diagnosis of SST treated in a tertiary pediatric hospital was performed. Furthermore, a 10-year review of the SST literature was completed. Three cases of SST at our institution were outlined. After reviewing the literature, 18 SST cases were identified. The mean age at diagnosis was 13.4 years, and the reported clinical presentations were abdominal or pelvic pain and menstrual irregularity. Seven patients had abnormal hormone tests or CA-125 levels. In approximately 30% of cases, conservative surgery was performed, preserving residual ovarian tissue. In conclusion, some preoperative findings may help in suggesting the presence of SST. However, definitive diagnosis can only be made by histopathological examination. It is important to consider this tumor because, given its benign behavior, a conservative approach is preferred, particularly in this age group.

Ovarian collision tumors are uncommon and reports of their radiological appearance are even less frequent. The present study reported the world's first case of an ovarian collision tumor consisting of an ovarian sclerosing stromal tumor and a mature cystic teratoma and its imaging presentation. When a cystic solid ovarian mass combined with ascites and elevated CA125 is encountered it is frequently diagnosed as a malignant tumor, but the present case was a benign tumor. Therefore, when encountering similar cases, clinicians should not limit the diagnosis to malignant tumors to avoid rashly expanding the surgery and causing unnecessary harm to the patient. The combination of computed tomography, magnetic resonance imaging and pathology findings presented in the current study enable radiologists to learn about this disease and further assist clinicians in developing the best treatment plan.

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.

Objectives This study was designed to evaluate the specific imaging features of ovarian sclerosing stromal tumor (SST), improve its accuracy as well as the specificity of imaging diagnosing, and prevent overestimation of malignancy to reduce unnecessary surgical procedures. Methods Eight patients with magnetic resonance imaging (MRI) and six with computed tomography (CT) images were analyzed in this retrospective observational study. All the cases were confirmed by postoperative pathological examination as those of ovarian SST. Imaging and pathological features were also evaluated. Results All the 14 masses displayed cystic and solid components with outer surface of tumors contained a capsular and complete smooth rim. Eight tumors of MRI exhibited “lake-island” sign on T2 weighted imaging (T2WI). Two of the 6 CT cases displayed a flaky calcification. One case showed as a multiloculated cystic mass with irregularly thickened septae and the tumor wall. The solid components in other 13 masses were comb- or wheel-like enhanced. After injection of contrast agent, the solid components in 8 cases (57.1%) appeared as early enhancement, whereas the other 6 cases (42.9%) appeared as progressive enhanced, and the cystic components of all the cases had no enhancement in the whole course. Vascular flow signals or/and marked enhancement of the blood vessels were found in 12 lesions (85.7%). Pathological examination demonstrated pseudolobule patterns, round to spindle shaped cells, collagenous areas, edematous hypocellular areas and prominent vasculatures. Conclusions The results demonstrated that MRI with “lake-island” signs on T2WI and MRI/CT dynamic enhancement could potentially play a critical role in facilitating appropriate diagnosis preoperatively.

Sclerosing stromal tumor (SST) is an extremely rare and distinctive sex cord stromal tumor, which occurs predominantly in the second and third decades of life. SSTs make up 2-6% of ovarian sex-cord stromal tumors. Due to the solid and distinct vascular structure of the tumor, it can be mistaken as a number of malignant ovarian tumors. As this specific neoplasm is very rare, it is not always possible to diagnose the tumor preoperatively with clinical and ultrasonographic findings. Furthermore, histopathological and immunohistochemical analysis does not always confirm the diagnosis. In this case report, clinical findings, histopathological features, and macroscopic appearance during laparoscopy of an SST are presented in a 20-year-old woman with pelvic pain. SST should be considered among the differential diagnosis of women with adnexal masses.

Sclerosing stromal tumour (SST) is a rare benign sex cord stromal tumour occurring in women in their second and third decades. Patients usually present with menstrual irregularity and pelvic pain. Microscopically, this tumour is characterized by epithelioid and spindle cells arranged in pseudolobules separated by areas with fibrous deposition of various amount. Presence of ‘staghorn like’ proliferating vasculature is the hallmark feature of this tumour. The main differential diagnoses are thecoma and fibroma. Immunohistochemistry can be used to differentiate these tumours. This relatively new entity should be kept in mind while reporting ovarian tumour in a young female. We have described SST in an 18 year old female in this case report. Bangladesh Journal of Medical Science Vol. 22 No. 03 July’23 Page : 712-717

Sclerosing stromal tumor (SST) of the ovary, which was first described by Chalvardjian and Scully in 1973, is a rare ovarian neoplasm, occurring predominantly in young women. The most common clinical symptom in patients with SST is menstrual irregularities. Microscopically, the tumor is characterized by the presence of pseudo-lobulated cellular areas, with a prominent tendency to sclerosis, marked vascularity and pronounced variation in cellular size and shape. In the current study, 2 cases of SST of the ovary are presented. These cases were confirmed by imaging, surgical and histological examination. No adjuvant therapy was administered to the patients and the two patients were disease-free with no imaging findings of recurrence or metastasis 24 months following surgery.