Clinical and ultrasound features of ovarian sclerosing stromal tumors: a large single-center case series
Introduction
Ovarian sclerosing stromal tumor (OSST) is a rare, benign neoplasm of sex cord-stromal origin, representing less than 6% of tumors in this category (1). It most frequently occurs in young women. The clinical presentation is often subtle, with many patients being asymptomatic; when symptoms do occur, they typically include menstrual irregularities or pelvic discomfort. A subset of OSSTs exhibits hormonal activity, estrogenic or androgenic effects, which can manifest as abnormal uterine bleeding, amenorrhea, or precocious puberty (2-4). Pathologically, OSST is characterized by a distinctive pseudolobular architecture, composed of alternating hypercellular and hypocellular areas within a collagenous stroma; immunohistochemistry is valuable for definitive diagnosis (5,6). Accurate preoperative recognition of OSST, though challenging, is critical for clinical decision-making. However, systematic descriptions of its sonographic features remain limited in the literature. This gap, combined with its nonspecific imaging appearance, often leads to misclassification as a malignant ovarian tumor. This diagnostic dilemma directly impacts clinical management, potentially leading to overly radical surgery (7,8).
Recognition of characteristic sonographic patterns can raise clinical suspicion of OSST. Diagnostic confidence is further enhanced when these imaging features are integrated with clinical clues such as hormonal symptoms—an approach particularly valuable in small ovarian sex cord-stromal tumors, where morphologic overlap with other ovarian tumors is common (9,10).
Based on a substantial single-center cohort, this study aimed to provide a comprehensive overview of the diverse sonographic presentations of OSST. The documented spectrum of imaging features, despite not enabling pathognomonic diagnosis due to overlaps with other entities, serves as an important reference to raise diagnostic suspicion among sonographers and clinicians. We present this article in accordance with the STROBE reporting checklist (available at https://qims.amegroups.com/article/view/10.21037/qims-2026-1-0052/rc).
Methods
This retrospective study was conducted at West China Second University Hospital (Sichuan University). It included 39 patients with a pathological diagnosis of OSST between January 2008 and October 2025. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Ethics Committee of West China Second University Hospital, Sichuan University (No. 2026036). The requirement for informed consent was waived in this retrospective study. Clinical data, ultrasound images, and pathological reports (including gross and histological findings) were systematically collected. Ultrasound examinations were performed using standard ultrasound systems by experienced sonographers, each with over 15 years of dedicated experience in gynecologic ultrasound, employing both transabdominal and transvaginal approaches with 5–9 and 2–5 MHz transducers, respectively. All imaging studies were independently reviewed by three experienced gynecologic sonographers (each with >15 years of experience) and also by a dedicated gynecologic radiologist with more than 15 years of experience. Discrepancies were resolved by consensus. The review focused on tumor characteristics: shape, margin definition, internal echogenicity, echo pattern, and the presence of cystic areas, calcifications, or posterior acoustic shadowing. Tumor vascularity was assessed using a semi-quantitative color score: 1 (no flow), 2 (minimal flow), 3 (moderate flow), and 4 (abundant flow). Intraoperative frozen section analysis was routinely performed in all patients. The final extent of surgery was determined based on the frozen section diagnosis, patient age, and fertility wishes. Final pathological diagnoses were confirmed by a gynecologic pathologist with over 15 years of experience in ovarian and sex cord-stromal tumors.
Results
A total of 39 OSST cases were diagnosed at our center between January 2008 and October 2025, with patient ages ranging from 4 to 73 years, with a median of 31.8 years. In this study, 79.5% (31/39) of the patients exhibited clinical symptoms, predominantly menstrual disorders or abnormal vaginal bleeding (56.4%). Other symptoms included infertility, pelvic or abdominal pain, abdominal distension, postmenopausal bleeding, and others. Clinically evident virilization was absent in all patients. Asymptomatic patients comprised 20.5% (8/39). Additionally, five cases were diagnosed during pregnancy, and seven cases showed elevated cancer antigen 125 (CA-125) levels (range, 46.2–147 U/mL). Among the three cases with postmenopausal bleeding, one was concurrent with cervical cancer and another with endometrial cancer. All 39 cases were unilateral, comprising 16 on the left side and 23 on the right. Surgical management varied according to patient age and clinical context: lesion excision alone was performed in 32 cases, unilateral adnexectomy in 4, and total hysterectomy with bilateral adnexectomy in 3 postmenopausal patients. No recurrence has been observed during follow-up to date. The clinical characteristics of the cohort are summarized in Table 1.
Table 1
| Characteristic | Data (n=39) |
|---|---|
| Age, years | 31.8 [4–73] |
| Clinical symptoms | 31 (79.5) |
| Menstrual disorders or abnormal vaginal bleeding | 22 (56.4) |
| Infertility | 3 (7.7) |
| Pelvic or abdominal pain | 3 (7.7) |
| Abdominal distension | 3 (7.7) |
| Postmenopausal bleeding | 2 (5.1) |
| Increased vaginal discharge | 1 (2.6) |
| Precocious puberty | 1 (2.6) |
| Urinary frequency | 1 (2.6) |
| Asymptomatic | 8 (20.5) |
| CA-125, U/mL | 92.0 [46.2–147] |
| Normal (<35) | 20 (51.3) |
| Elevated (≥35) | 7 (17.9) |
| Not tested | 12 (30.8) |
| Surgical approach | |
| Tumor excision | 32 (82.1) |
| Unilateral adnexectomy | 4 (10.3) |
| Total hysterectomy and bilateral adnexectomy | 3 (7.7) |
| Recurrence within follow-up period | 0 (0) |
Data are presented as n (%) or median [range]. CA-125, cancer antigen 125.
In our cohort, preoperative ultrasound detected adnexal masses in all 39 cases, with a median maximum diameter of 67 mm (range, 24–154 mm). A total of 13 tumors (33.3%) measured <50 mm, including 2 (5.1%) that were ≤25 mm. All lesions (100%) presented as well-defined, round, or ovoid masses. The internal echogenicity was heterogeneous in 32 cases (82.1%) and homogeneous in 7 (17.9%). Morphologically, 18 tumors (46.2%) were purely solid, 19 (48.7%) were solid with cystic areas, and 2 (5.2%) were completely cystic (one unilocular and one multilocular). Posterior acoustic shadowing and calcifications were each present in 2 cases (5.1%). Most lesions demonstrated moderate vascularity (color score 3 in 31/39). The median resistance index (RI) was 0.45 (range, 0.24–0.65). Peripheral regular vascularity was observed in 25 tumors (64.1%). Among the 39 OSSTs, contrast-enhanced ultrasound (CEUS) was performed in a single case. The examination demonstrated early, intense hyperenhancement with a centripetal filling pattern; in the late phase, a persistent peripheral ring of enhancement was noted. Regarding Ovarian-Adnexal Reporting and Data System (O-RADS) classification, the majority of tumors were categorized as category 4 (33/39, 84.6%) or 5 (3/39, 7.7%). Two lesions showing acoustic shadowing were classified as O-RADS 3, and one unilocular cystic lesion was assessed as O-RADS 2. Among the 39 OSSTs, preoperative ultrasound suggested fibroma in 2 cases (2/39), broad ligament leiomyoma in 1 case (1/39), and germ cell tumor in 1 case (1/39), whereas the remaining 35 cases (89.7%) were reported as indeterminate ovarian tumors without a definitive benign or malignant diagnosis. The sonographic characteristics of the cohort are summarized in Table 2, with representative images shown in Figure 1.
Table 2
| Feature | Data |
|---|---|
| Maximum lesion diameter, mm | 67 [24–154] |
| ≥50 | 26 (66.7) |
| >25, <50 | 11 (28.2) |
| ≤25 | 2 (5.1) |
| Location | |
| Right ovary | 23 (59.0) |
| Left ovary | 16 (41.0) |
| Round or ovoid shape | 39 (100.0) |
| Regular margins | 39 (100.0) |
| Internal echogenicity | |
| Homogeneous | 7 (17.9) |
| Heterogeneous | 32 (82.1) |
| Morphology | |
| Solid | 18 (46.2) |
| Solid with cystic area | 19 (48.7) |
| Unilocular cyst with smooth inner wall | 1 (2.6) |
| Multilocular cyst with smooth inner wall | 1 (2.6) |
| Shadowing | 2 (5.1) |
| Calcification | 2 (5.1) |
| Color score | |
| 1 | 1 (2.6) |
| 2 | 4 (10.3) |
| 3 | 31 (79.5) |
| 4 | 3 (7.7) |
| Regular peripheral vascularity | 25 (64.1) |
| Resistance index | 0.45 [0.24–0.65] |
| O-RADS category | |
| 2 | 1 (2.6) |
| 3 | 2 (5.1) |
| 4 | 33 (84.6) |
| 5 | 3 (7.7) |
| Preoperative ultrasound diagnosis | |
| Fibroma | 2 (5.1) |
| Broad ligament leiomyoma | 1 (2.6) |
| Germ cell tumor | 1 (2.6) |
| Indeterminate whether benign or malignant | 35 (89.7) |
Data are presented as n (%) or median [range]. Color scoring system: 1, no flow; 2, minimal flow; 3, moderate flow; 4, abundant flow. O-RADS, Ovarian-Adnexal Reporting and Data System.
Discussion
OSST is a rare, benign sex cord-stromal neoplasm that poses significant diagnostic challenges due to its nonspecific clinical and imaging presentation. Our study presents the clinical and sonographic features of 39 pathologically confirmed OSST cases, which, to our knowledge, represents one of the largest single-center series with comprehensive sonographic documentation reported to date. The exceptional value of this series lies not only in its size but also in its broad demographic span and the inclusion of particularly rare subgroups. Our cohort encompasses patients from 4 to 73 years of age, providing a unique panoramic view of the disease across the lifespan. We included distinct clinical subgroups that are seldom captured in smaller reports: a premenarchal child, pregnant patients, and postmenopausal women. The substantial number of cases enables a more robust and detailed characterization of the tumor’s sonographic spectrum than previously possible, moving beyond anecdotal descriptions to identify recurring patterns and variations.
Although OSST is classically described as a tumor of young women, our cohort confirms and extends this profile. The case of a 4-year-old girl with precocious puberty further supports the rare but established association between OSST and endocrine effects in very young patients (4,11,12). Therefore, in the diagnostic evaluation of a prepubertal child presenting with isolated peripheral precocious puberty alongside an ovarian mass, OSST warrants inclusion in the differential diagnosis.
Our series also provides insights into OSST in postmenopausal women—a distinctly rare scenario (3,13,14). Notably, although earlier reports of postmenopausal OSST did not describe concomitant malignancy, our series expands this profile by detailing two such cases. All three postmenopausal patients presented with bleeding, which was directly attributed to concurrent gynecologic pathology: endometrial carcinoma, cervical carcinoma, and endometrial hyperplasia, respectively. This triad highlights a critical clinical challenge: the estrogenic potential of OSST and the complexity of differential diagnosis. When an adnexal mass coexists with an endometrial carcinoma, a key preoperative consideration is whether the ovarian lesion represents a metastatic deposit. It is plausible that hormonally active tumors contributed to the endometrial changes. Consequently, in a postmenopausal woman with bleeding and an adnexal mass, OSST should be considered not only in the differential diagnosis of the mass itself but also as a potential hormonal contributor to the endometrial pathology.
The five cases of OSST co-occurring with pregnancy in our series further delineate the tumor’s behavior. All patients were asymptomatic, with tumors discovered incidentally. Two patients underwent tumor resection at 15 and 18 weeks of gestation due to larger tumor size (with maximum diameters of 102 and 93 mm, respectively), whereas the remaining three underwent tumor resection concurrently with cesarean delivery at term. Management followed established principles: asymptomatic masses were safely managed until delivery, whereas surgery during the mid-trimester was a viable option for larger tumors. All pregnancies culminated in favorable outcomes, reinforcing that with appropriate timing, OSST does not adversely affect pregnancy (15).
Ultrasound remains the primary imaging modality for initial evaluation. In our series, OSST typically presented as a well-circumscribed, round, or ovoid mass, and was invariably unilateral, though rare bilateral cases have been documented (16,17). The vast majority (37/39, 94.9%) exhibited predominantly hypoechoic solid components. Morphologically, tumors presented as purely solid (18/39, 46.2%) or solid-cystic (19/39, 48.7%). Two distinct patterns were also observed: one unilocular cystic and one multilocular (honeycomb-like) lesion. This spectrum corresponds to the “cystic-solid” or “mainly solid” patterns identified in prior computed tomography/magnetic resonance imaging (CT/MRI) studies (18,19). Notably, both calcifications and posterior acoustic shadowing were uncommon, each present in only two cases (5.1%) (17,20). A majority of OSSTs (25/39, 64.1%) exhibited a characteristic pattern of regular peripheral vascularity. This finding has also been captured on sonograms in a number of previously reported cases (21,22). Furthermore, CEUS findings in our study—specifically early hyperenhancement with centripetal progression and a late peripheral ring sign—reflect the same dynamic vascular pattern long recognized on contrast-enhanced CT and MRI, the established reference modalities for further characterization of OSST (18,19,23,24). However, given that CEUS was performed in only one patient, these observations remain anecdotal and should be interpreted with caution. Larger prospective studies are needed to determine whether this enhancement pattern is a consistent feature of OSST on CEUS. Given these limitations, further evaluation with contrast-enhanced CT or MRI remains essential for diagnostic confirmation and surgical planning.
In our cohort, the majority of OSSTs were classified as O-RADS category 4 (33/39, 84.6%) or 5 (3/39, 7.7%), collectively indicating an intermediate to high risk of malignancy. Only three cases with clearly benign features (a completely cystic lesion or those with acoustic shadowing) were categorized as O-RADS 2 or 3 (25,26). This pattern of over-classification highlights a significant limitation of the current O-RADS system for OSST and underscores the need for more nuanced stratification strategies for such masses. Although most OSSTs were classified as O-RADS 4 or 5, all patients with fertility potential underwent conservative surgery guided by intraoperative frozen section. This underscores that O-RADS alone should not dictate surgical extent; intraoperative pathology remains essential to avoid overtreatment in young women with this benign tumor.
The differential diagnosis primarily includes ovarian fibroma, subserosal or broad ligament leiomyoma, and hypervascular tumors such as hemangioma (27). A possible distinguishing feature is that most OSSTs lack significant posterior acoustic shadowing but demonstrate prominent peripheral vascularity. This profile differs from an ovarian fibroma, characterized by marked shadowing and minimal vascularity. Interestingly, there are pathological reports of OSST coexisting with fibroma or other tumors, leading to the hypothesis that OSST may represent a transitional tumor (28,29). Our series provides intriguing imaging support for this concept: one tumor in our cohort presented with typical fibroma features (including marked acoustic shadowing) prior to pregnancy, which transformed into a shadowing-free appearance during gestation, morphologically mirroring the classic OSST pattern. Although the vascular pattern of a leiomyoma can resemble OSST, its diagnosis hinges on identifying its anatomical continuity with the uterus. A hemangioma may also show rich peripheral flow but typically exhibits more extensive and diffuse internal vascularity (30). In contrast, common ovarian malignancies rarely display the organized peripheral vascular rim seen in OSST. This nuanced analysis of both morphology and vascular architecture provides critical diagnostic information that supplements the O-RADS classification.
Preoperative evaluation may reveal ascites (potentially suggestive of Meigs’ syndrome) or elevated serum CA-125, both of which can raise suspicion of malignancy (8,14,31). However, it is important to recognize that the majority of OSSTs are associated with neither feature (32,33). This is supported by our series, in which ascites was absent and only 7 of 39 patients (17.9%) exhibited elevated CA-125 levels. Given its benign nature, fertility-sparing surgery is the management cornerstone in young patients (34). In our cohort, management was tailored to age and fertility status: all premenopausal patients (n=36) underwent fertility-sparing procedures (lesion excision or unilateral adnexectomy), whereas three postmenopausal women underwent hysterectomy with bilateral adnexectomy. No recurrences were observed, confirming that complete excision is curative and that radical surgery is unnecessary.
Emerging technologies such as radiomics and machine learning have shown promise in gynecologic oncology (35-37), and structured training has been demonstrated to improve ultrasound diagnostic skills (38). Although these approaches have not yet been applied to OSST due to its rarity and the lack of large imaging datasets, they hold future potential for distinguishing OSST from morphologically overlapping tumors and for disseminating pattern recognition expertise. These considerations underscore the need for multicenter prospective collaborative efforts.
Conclusions
OSST is a diagnostically challenging benign tumor that should be considered in females of all ages presenting with a unilateral, solid-cystic, hypervascular adnexal mass. It is frequently over-classified by ultrasound systems, necessitating careful integration of clinical, hormonal, and imaging data. Awareness of its variable presentation and benign course is crucial to guide conservative surgical management and avoid unnecessary radical procedures.
Acknowledgments
None.
Footnote
Reporting Checklist: The authors have completed the STROBE reporting checklist. Available at https://qims.amegroups.com/article/view/10.21037/qims-2026-1-0052/rc
Data Sharing Statement: Available at https://qims.amegroups.com/article/view/10.21037/qims-2026-1-0052/dss
Funding: None.
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://qims.amegroups.com/article/view/10.21037/qims-2026-1-0052/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. The study was conducted in accordance with the Declaration of Helsinki and its subsequent amendments. The study was approved by the Ethics Committee of West China Second University Hospital, Sichuan University (No. 2026064). Informed consent was waived in this retrospective study.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
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