No approved-drug candidate for ovarian sex cord-stromal tumor is corroborated in the literature DeepSearch retrieved. Some conditions are managed with non-pharmacological care — a device, surgery or physical therapy — rather than a medicine; that may be the case here, or the literature we found may simply be too sparse yet to support a drug-repurposing angle.
RCSB Protein Data Bank · entry 7XW2 · 3.04 Å · ligand none (apo structure). Experimental structure, not a prediction.
Ovarian sex cord-stromal tumours represent about 7% of all primary ovarian tumours, a figure from a 2015 review. That same review states the majority present as low-grade disease with a nonaggressive clinical course, especially in younger patients, and are linked to hormone-mediated syndromes because the tumour cells produce androgens, oestrogens, or corticoids. The 2015 paper also notes the World Health Organization classification was recently revised, regrouping tumours into pure stromal, pure sex cord, and mixed sex cord-stromal entities, and removing stromal luteoma, stromal tumour with minor sex cord elements, and gynandroblastoma as separate categories.
A 1993 article, despite its title about dialogue managers, claims to focus on recent advances in the molecular underpinnings of ovarian sex cord-stromal tumours. It states that integrating genetic information with morphology and immunohistochemistry has clinical significance for refining diagnostic and prognostic stratifications and for genetic counselling. No specific molecular findings, patient numbers, or survival data are given in that abstract.
A 2024 review, part I on pure ovarian stromal tumours, describes them as time-honoured but still frequently challenging to diagnose, with unusual morphologic appearances that cause a disproportionate number of differential diagnostic problems. It mentions variant immunohistochemical profiles and specific molecular and syndromic associations, but provides no concrete response rates, survival figures, or sample sizes. The review notes that current knowledge is still based in significant part on the contributions of two pathologists, Dr Robert Meyer and Dr Robert E. Scully.
What is missing from the published literature on these tumours is any controlled trial data, any report of drug response rates, and any prospective study that stratifies patients by molecular subtype. No abstract reports a treatment outcome, a survival benefit, or a repurposed drug. The evidence base remains descriptive and historical, lacking the financial support and trial infrastructure needed to move from morphological classification to actionable therapy.
Retrieved by DeepSearch across 234,678,978 indexed works and resolved on OpenAlex — ranked by citations, including the results that did not work.
Diagnostic and Interventional Radiology · 2015 · 152 citations · open access
Sex cord-stromal tumors of the ovary: a comprehensive review and update for radiologists
AbstractOvarian sex cord-stromal tumors are infrequent and represent approximately 7% of all primary ovarian tumors. This histopathologic ovarian tumor group differs considerably from the more prevalent epithelial ovarian tumors. Although sex cord-stromal tumors present in a broad age group, the majority tend to present as a low-grade disease that usually follows a nonaggressive clinical course in younger patients. Furthermore, because the constituent cells of these tumors are engaged in ovarian steroid hormone production (e.g., androgens, estrogens, and corticoids), sex cord-stromal tumors are commonly associated with various hormone-mediated syndromes and exhibit a wide spectrum of clinical features ranging from hyperandrogenic virilizing states to hyperestrogenic manifestations. The World Health Organization sex cord-stromal tumor classification has recently been revised, and currently these tumors have been regrouped into the following clinicopathologic entities: pure stromal tumors, pure sex cord tumors, and mixed sex cord-stromal tumors. Moreover, some entities considered in the former classification (e.g., stromal luteoma, stromal tumor with minor sex cord elements, and gynandroblastoma) are no longer considered separate tumors in the current classification. Herein, we discuss and revise the ultrasonography, computed tomography, and magnetic resonance imaging characteristics of the different histopathologic types and clinicopathologic features of sex cord-stromal tumors to allow radiologists to narrow the differential diagnosis when facing ovarian tumors.
https://doi.org/10.5152/dir.2015.34414International Journal of Gynecological Pathology · 2006 · 108 citations
Recent Advances in the Pathology and Classification of Ovarian Sex Cord-Stromal Tumors
AbstractIn recent years, our knowledge of ovarian sex cord-stromal tumors has increased, and their classification has evolved. In this review, recent advances in the classification and pathology of ovarian sex cord-stromal tumors are discussed, and the controversy regarding the classification of sex cord tumor with annular tubules is addressed. The current classification is built on those of the past, and future classifications should improve on what is now in place incorporating new knowledge from more sophisticated clinicopathologic studies and advanced molecular techniques. This review emphasizes articles written in the 21st century as well as those that have significantly advanced our knowledge of sex cord-stromal tumors in past decades. The tumors in this group occur over a wide age range and are often unilateral. In difficult cases, immunocytochemistry provides improved diagnostic accuracy. The most useful immunohistochemical marker for their identification is alpha-inhibin, which is positive in most neoplasms in the sex cord-stromal group. The article concludes with a section discussing the pathogenesis of sex cord-stromal tumors.
https://doi.org/10.1097/01.pgp.0000192271.22289.e6National Conference on Artificial Intelligence · 1993 · 10 citations
A method for development of dialogue managers for natural language interfaces
AbstractThis article focuses on the recent advances in ovarian sex cord-stromal tumors, predominantly in the setting of their molecular underpinnings. The integration of genetic information with morphologic and immunohistochemical findings in this rare subset of tumors is of clinical significance from refining the diagnostic and prognostic stratifications to genetic counseling.
https://doi.org/10.1016/j.path.2022.02.004Discover Oncology · 2024 · 4 citations · open access
Current status of treatment for malignant ovarian sex cord-stromal tumors
AbstractMalignant ovarian sex cord-stromal tumors belong to a specific type of ovarian epithelial carcinoma, and their prognosis is related to the kind of pathology. Unlike epithelial ovarian cancer, most malignant ovarian sex cord-stromal tumors are low-grade malignant and have a good prognosis. Still, they have a tendency to recur in the long term, and the mortality rate of patients after recurrence is high. Surgery is the preferred treatment for malignant ovarian sex cord-stromal tumors with chemotherapy, and other comprehensive treatments. Recurrent and metastatic malignant ovarian sex cord-stromal tumors usually require systemic adjuvant chemotherapy, and hormonal or targeted therapy may be attempted on an individualized basis in cases where chemotherapy fails.
https://doi.org/10.1007/s12672-024-01687-6Advances in Anatomic Pathology · 2024 · 3 citations
Sex Cord-Stromal Tumors of the Ovary: An Update and Review. Part II — Pure Sex Cord and Sex Cord-Stromal Tumors
AbstractWe review the time honored but still frequently challenging features of ovarian sex cord-stromal tumors and also emphasize new developments, including unusual morphologic appearances that, despite the relative rarity of many of the tumors, result in a disproportionate number of differential diagnostic problems, variant immunohistochemical profiles, and specific molecular and syndromic associations. These neoplasms are also of historical interest as current knowledge is still based in significant part to the contributions of 2 giants of gynecologic pathology, Dr Robert Meyer and Dr. Robert E. Scully. In part I, we reviewed the pure ovarian stromal tumors. Now, in part II, we present the major clinical, pathologic, and genomic features of pure sex cord and sex cord-stromal tumors.
https://doi.org/10.1097/pap.0000000000000436Advances in Anatomic Pathology · 2024 · 2 citations
Sex Cord–Stromal Tumors of the Ovary: An Update and Review. Part I — Pure Ovarian Stromal Tumors
AbstractIn two separate reviews, we review the time-honored but still frequently challenging features of ovarian sex cord-stromal tumors, and also emphasize new developments including unusual morphologic appearances that, despite the relative rarity of many of the tumors, result in a disproportionate number of differential diagnostic problems, variant immunohistochemical profiles, and specific molecular and syndromic associations. These neoplasms are also of historical interest as current knowledge is still based in significant part on the contributions of 2 giants of gynecologic pathology, Dr Robert Meyer and Dr Robert E. Scully. In part I, we present the major clinical, pathologic, and genomic features of the pure ovarian stromal tumors including comments on differential diagnosis and briefly note significant historical contributions. In part II we will discuss pure sex cord and sex cord-stromal tumors.
https://doi.org/10.1097/pap.0000000000000435Oncology Times · 2023 · 0 citations
The Impact of Surgery & Chemo in Treating Sex Cord-Stromal Tumors
AbstractGynecologic Cancer: Gynecologic CancerA recent analysis suggests that the use of chemotherapy in the first-line or relapse setting has no impact on survival among patients with sex cord-stromal tumors (SCST). These findings, which were presented during the 2023 ESMO Sarcoma and Rare Cancers Congress, also showed that only surgery—and its quality—led to a progression-free survival benefit for ovarian SCST in the first-line and relapse settings (Abstract 130). “Ovarian sex cord-stromal tumors are very rare non-epithelial tumors that represent 3-5 percent of ovarian neoplasms,” according to study author Hélène Vanacker, MD, in the Medical Oncology Department at the Centre Léon Bérard in Lyon, France, who noted that it's a “complex histological diagnosis requiring systematic review and potential molecular analysis.” These tumors are classified according to the cell types of origin, she explained. The most common subtypes are adult granulosa cell tumors and Sertoli-Leydig cell tumors, with an age of diagnosis of menopause/perimenopause and young women, respectively. “Surgery is the cornerstone of treatment,” Vanacker noted, “with fertility-sparing surgery for early stage and young patients as compared to radical surgery for advanced disease or FIGO IC2-3-II.” Depending on tumor type and prognostic factors, the postoperative chemotherapy options include bleomycin etoposide carboplatin (BEP) or carboplatin/paclitaxel. In the relapse setting, repeat surgical resections are considered when feasible. There are a number of challenges associated with SCSTs, according to Vanacker. This includes a need for accurate diagnosis, as well as defining prognosis and best standard of care for these patients. The research team initiated the current study to address these gaps in care. Methods & Results The researchers collected and analyzed data from 13 TMRG network centers. A total of 469 adult patients with malignant SCST undergoing upfront surgery were enrolled. The inclusion period was from 2011 to July 2015. Vanacker and colleagues performed progression-free and overall survival analyses. Among enrolled patients, 65.2 percent had early-stage disease (Figo IA-IB), according to the study authors. Stage I disease was reported in 87.5 percent of patients. Stage III and IV disease was seen in 6.7 percent of participants. Seventy-five percent (n=359) of included patients were diagnosed with adult Granulosa cell tumors. A total of 13 percent (n=61) were diagnosed with Sertoli-Leydig cell tumors, and 10.4 percent (n=49) had another rare subtype. Median follow-up was 6.4 years and 154 (32.8%) patients developed first recurrence during that time, according to the study authors. Of those individuals, second and third recurrences occurred in 82 (17.5%) and 49 (10.4%), respectively. Vanacker and colleagues reported that 67 (14.7%) patients were administered adjuvant chemotherapy at initial diagnosis. Of those patients, 74.6 percent received BEP and 15.9 percent were administered carboplatin-paclitaxel. When looking at the relapse setting, data showed that 125 (92.6%), 51 (71.8%), and 25 (59.5%) patients had surgery in the first, second, and third relapse, respectively. Seventy-nine (58.5%), 20 (28.2%), and 10 (23.8%) patients received perioperative chemotherapy in first, second, and third relapse, respectively. Vanacker noted that nine (7%), 12 (17%), and 13 (31%) patients received chemotherapy alone in first, second, and third relapses. The quality of the surgery was very good, according to Vanacker, with 97 percent of patients undergoing complete (CC0) surgery. Eight (1.7%) patients received incomplete surgery—two patients with Stage II and six patients with Stage III/IV disease. Survival analyses revealed that first-line therapy, age less than 70 years, FIGO stage, and complete surgery were associated with longer progression-free survival, according to Vanacker and colleagues. Additionally, chemotherapy had no impact on progression-free survival in early-stage disease (FIGO I-II). The study authors observed a similar progression-free survival when using BEP or other chemotherapy regimens in the first-line setting. However, they reported that at relapse BEP use improved survival when compared with all other chemotherapy regimens. Data revealed a connection between complete surgery and statistically prolonged progression-free survival in the case of recurrence. Vanacker noted that perioperative chemotherapy did not have an impact on progression-free survival. Study Conclusions This research sheds light on the role of chemotherapy and surgery among patients with these rare tumors; however, further study is needed to confirm these findings. Vanacker and colleagues underscore the need for randomized clinical trials to confirm that chemotherapy use has no impact on survival in the first-line and relapse setting of sex cord-stromal tumors. Good quality surgery demonstrates a progression-free survival benefit in the first line, as well as in first and second relapses, Vanacker concluded during her ESMO presentation. However, she noted that, at relapse, incomplete surgery has comparable median progression-free survival to the absence of surgery. “Chemotherapy for Figo Stage IC and II, at baseline or in relapse after surgery, did not improve progression-free survival,” she said. “BEP chemotherapy did not seem to improve efficacy in our cohort compared to other chemotherapy regimens in first-line setting. We need dedicated randomized trials, especially for advanced stage and relapse, to definitely answer the role of chemotherapy after complete surgery.” Catlin Nalley is a contributing writer.
https://doi.org/10.1097/01.cot.0000944532.83079.52Obstetrical & Gynecological Survey · 2009 · 0 citations
Improvement of Survival in Sex Cord Stromal Tumors: An Observational Study With More Than 25 Years Follow-Up
AbstractSex cord stromal tumors are rare malignant ovarian malignancies that have an overall favorable prognosis and are characterized by early diagnosis, slow growth, and late recurrence. The mainstay of initial management is surgery. No data support postoperative adjuvant treatment for patients with International Federation of Gynecology and Obstetrics (FIGO) stage I, which represent the majority of such tumors. In the last 3 decades, major changes in treatment of sex cord stromal tumors have occurred with the use of more radical surgery, abandonment of radiotherapy, marked decrease in utilization of adjuvant chemotherapy, and the establishment of new chemotherapy regimens, especially those based on platinum. This population-based cohort study was an epidemiologic historic overview that highlighted the importance of FIGO-stage and changes in treatment strategies to improved rates of survival over a study period of 28 years among a population of patients with ovarian sex cord stromal tumors. The study population was comprised of 145 women with a primary sex cord stromal tumor. Data were obtained from the Munich Cancer Registry in Munich, Germany. Two time periods were examined. The first study period was 1978 to 1987 and the second was 1988 to 2005. The Kaplan-Meier method was used to calculate unadjusted estimates of overall survival. Relative survival (an estimate of disease-specific survival) was calculated as the ratio of the observed to expected survival rate. Multivariate analysis with a Cox regression model was used to determine whether age, disease stage (FIGO), postoperative residual tumor, and chemotherapy were independent prognostic factors for infection. The proportion of women with FIGO-stage I increased from 42.1% in the first study period to 77.8% in the second. Five of 10-year overall survival improved from 55.8%/42.8% (relative survival 58.6%/49.2%) in the first study period to 89.1%/78.3% (relative survival: 92.7%/85.2%) in the second study period. Adjustment for age and FIGO-stage showed that the hazards ratio following surgery in patients with residual tumor was 3.3 (95% confidence interval, 1.5–7.0) compared to those without residual tumor, whereas women without chemotherapy had a hazards ratio of 2.2 (95% confidence interval, 1.2–4.2) in comparison to those with chemotherapy. These findings suggest that the 30% improvement in survival over the 28-year study period result from a stage-shift toward earlier disease stages and advances in treatment such as more radical surgery without residual tumor. Surgery remains the mainstay of treatment. Adjuvant chemotherapy is under active investigation but appears to provide no benefit.
https://doi.org/10.1097/01.ogx.0000351676.77800.5eDisease module: DeepOracle (Open Targets). Structures: RDKit from PubChem SMILES. Literature: retrieved by DeepSearch across 234,678,978 indexed works (targeted per-candidate search), resolved on OpenAlex.
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