These are reviewed studies whose abstracts concern Ovarian Sarcoma. Each describes only what that study reported. This is not a claim by OncoForge that any compound helps or harms Ovarian Sarcoma. Most are early lab, animal, or small human studies, and findings often conflict.
Case reportMixed resultsLimited evidenceTier 3 · early humann = 1
Journal of Indian Association of Pediatric Surgeons · Sep 2023 · case report
ovarian sarcoma
This single-case report describes an 8-year-old girl presenting with a large abdominal mass and cachexia. Initial tests suggested a germ cell tumor (raised AFP) but biopsy and IHC indicated a spindle cell, non-rhabdomyosarcoma ovarian sarcoma; because pediatric ovarian non-rhabdomyosarcoma is not reported, she received a rhabdomyosarcoma neoadjuvant chemotherapy regimen and had a good response, followed by complete surgical excision and radiotherapy. The authors note that overall outcomes for the disease are dismal and more data are needed to guide treatment.
Studied with: surgery, radiotherapy.
Key findings
- Patient: 8-year-old girl with large abdominal mass and cachexia.
- Raised alpha-fetoprotein (AFP) levels suggested a germ cell tumor.
- Tru-cut biopsy reported a spindle cell tumor.
- Immunohistochemistry (IHC) suggested a non-rhabdomyosarcoma.
- Because pediatric ovarian non-rhabdomyosarcoma was not reported, a rhabdomyosarcoma neoadjuvant chemotherapy regimen was started.
- Good response was noted to neoadjuvant chemotherapy.
- Treatment was followed by complete surgical excision of the tumor and radiotherapy.
- Authors state that the overall outcome of the disease is dismal and call for more data.
Limitations: Single-patient case report (n=1).; No long-term follow-up or outcome details for this patient are provided.; No chemotherapy regimen details, doses, or schedules reported.; No control or comparison group.; Limited pathology and molecular diagnostic detail reported in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
Case reportReported negativeLimited evidenceTier 3 · early humann = 1
Medicina (Kaunas, Lithuania) · Oct 2022 · case report
Doxorubicinhigh-grade endometrioid stromal sarcoma of the ovarymature cystic teratoma (ovary) This is a single-patient case report of a 62-year-old woman whose ovarian mature cystic teratoma underwent malignant transformation to a high-grade endometrioid stromal sarcoma (FIGO stage IIIC). She underwent debulking cytoreductive surgery followed by three courses of adjuvant doxorubicin, but the cancer recurred 3 months after surgery and the patient died of progressive disease. Imaging showed invasive features on MRI and high FDG uptake on PET/CT that suggested malignancy.
Reported effects: age 62, n=1 · symptom_duration 6 mo, n=1 · +4 more
Studied with: debulking cytoreductive surgery.
Key findings
- A 62-year-old woman presented with 6 months of lower abdominal pain.
- Magnetic resonance imaging showed two adnexal masses with fat components consistent with mature cystic teratomas; the left mass had an eccentric thick rim with irregular invasion of the uterus suggestive of malignancy.
- PET/CT demonstrated high fluorodeoxyglucose uptake in the corresponding area.
- The patient underwent debulking cytoreductive surgery and was diagnosed with FIGO stage IIIC high-grade endometrioid stromal sarcoma arising from a mature cystic teratoma.
- After surgery the patient received adjuvant chemotherapy with three courses of a doxorubicin regimen (dose not specified).
- The cancer recurred 3 months after surgery, and the patient died of progressive disease.
Limitations: Single-patient case report (n=1), limiting generalizability.; No chemotherapy dose, schedule details, or response metrics provided.; Short interval to recurrence and limited follow-up information.; No control or comparator; observational description only..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
Human · observationalInconclusiveModerate evidenceTier 3 · early humann = 31727
Gynecologic oncology · Dec 2021 · registry-based comparative study (Korea Central Cancer Registry, 1999-2017)
primary ovarian sarcomaepithelial ovarian cancer
This population-based registry study compared incidence and 5-year survival of primary ovarian sarcoma (n=1361) versus epithelial ovarian cancer (n=30,366) using Korea Central Cancer Registry data from 1999–2017. Incidence (ASR) was 0.22 per 100,000 for primary ovarian sarcoma versus 4.75 per 100,000 for epithelial ovarian cancer (ASR ratio 21.94). Five-year overall survival was 64.0% for primary ovarian sarcoma and 61.5% for epithelial ovarian cancer (p = 0.6030), a difference that was not statistically significant. Among pure sarcoma subtypes, 5-year overall survival was 85.0% for fibrosarcoma, 76.7% for liposarcoma, and 72.7% for stromal cell sarcoma (p < 0.0001).
Reported effects: ASR epithelial ovarian cancer 4.75 · ASR primary ovarian sarcoma 0.22 · +5 more
Key findings
- ASR for epithelial ovarian cancer = 4.75 per 100,000 women.
- ASR for primary ovarian sarcoma = 0.22 per 100,000 women.
- ASR ratio = 21.94 with no significant change in ASR during the study period.
- Five-year overall survival: primary ovarian sarcoma 64.0% vs epithelial ovarian cancer 61.5% (p = 0.6030), difference not statistically significant.
- Among pure sarcomas, 5-year overall survival: fibrosarcoma 85.0%, liposarcoma 76.7%, stromal cell sarcoma 72.7% (p < 0.0001).
Limitations: Observational registry data subject to confounding and limited ability to infer causation.; Abstract does not report adjusted analyses or control for potential prognostic confounders (e.g., stage, treatment).; Registry-based dataset may lack detailed clinical, pathologic, or treatment information in the abstract.; Single-country (Korea) data may limit generalizability to other populations.; Very low incidence of primary ovarian sarcoma may limit some subgroup analyses despite overall sample size..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Human · observationalMechanismReported positiveLimited evidenceTier 3 · early humann = 3
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · Jan 2020 · case series (3 cases) with comprehensive literature review
ovarian sarcomaperitoneal sarcomaintracranial sarcomapleuropulmonary blastomagenitourinary embryonal rhabdomyosarcomaanaplastic sarcoma of the kidney
The authors report three pediatric sarcoma cases (ovarian with germline DICER1 mutation; metastatic peritoneal and primary intracranial with somatic DICER1 mutations) and performed a literature review of DICER1-associated sarcomas. The review (including 83 cases) shows a consistent heterogeneous histologic pattern similar to pleuropulmonary blastoma across sites. They recommend that this distinctive histology should prompt review of family history and DICER1 mutation testing to enable genetic counseling and imaging surveillance.
Reported effects: cases_reported 3, n=3 · literature_review_count 83, n=83
Key findings
- Reported three pediatric sarcomas associated with DICER1 mutations: a germline DICER1-associated ovarian sarcoma (5-year-old female), a somatic DICER1-associated metastatic peritoneal sarcoma (16-year-old female), and a somatic DICER1-associated primary intracranial sarcoma (4-year-old male).
- Comprehensive literature review including 83 DICER1-associated sarcomas demonstrates a consistent histologic pattern that mimics pleuropulmonary blastoma regardless of site.
- Characteristic histologic features include undifferentiated small round blue cells, poorly differentiated spindle cells, and large bizarre pleomorphic (anaplastic) cells, often with rhabdomyoblastic and/or chondroid differentiation and occasional bone/osteoid formation.
- The authors state that this heterogeneous histologic pattern should prompt detailed family-history review and DICER1 mutation analysis, facilitating genetic counseling, caregiver education, and imaging-based surveillance.
Limitations: Small case series (n=3) reported from a retrospective/case-report design; Descriptive literature review without systematic meta-analysis or pooled quantitative synthesis; No functional experiments in this report to demonstrate biological causality between DICER1 variants and the described histology; Findings may be subject to reporting/selection bias and limited generalizability.
Expands the phenotypic spectrum of DICER1-associated tumors and highlights a characteristic histology that may indicate underlying DICER1 mutations.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed · Full text
Human · observationalMixed resultsLimited evidenceTier 3 · early humann = 1258
European journal of cancer (Oxford, England : 1990) · Sep 2019 · population-based registry study (retrospective cohort)
non-epithelial ovarian tumoursovarian germ cell tumoursovarian sex cord-stromal tumoursovarian sarcomas
This population-based study used the Netherlands Cancer Registry to describe incidence, treatment, and overall survival for women with non-epithelial ovarian malignancies diagnosed 1989–2015. The authors report 1,258 cases (752 germ cell tumours, 341 sex cord-stromal tumours, 165 sarcomas); most underwent surgery, about 31% received systemic therapy, and 3% radiotherapy. Five-year overall survival increased over the study period for germ cell tumours (from 73% to 88%, p = 0.03), sex cord-stromal tumours (64% to 81%, p = 0.57) and sarcomas (20% to 29%, p = 0.14).
Reported effects: 5-year overall survival (GCTs) 88%, p=0.03, n=752 · 5-year overall survival (SCSTs) 81%, p=0.57, n=341 · +1 more
Key findings
- Total 1,258 malignant non-epithelial ovarian tumours identified: 752 GCTs (60%), 341 SCSTs (27%), 165 sarcomas (13%).
- European age-standardised incidence rate (ESR) per 100,000 person-years: GCTs 0.4, SCSTs 0.2, sarcomas 0.1.
- About 97% of patients underwent surgical resection of the primary tumour, 31% received systemic treatment and 3% received radiotherapy.
- Five-year overall survival improved between the late 1980s and 2015: GCTs rose from 73% to 88% (p = 0.03), SCSTs from 64% to 81% (p = 0.57), and sarcomas from 20% to 29% (p = 0.14).
- Incidence of malignant GCTs and SCSTs did not significantly change over the study period; sarcomas remain extremely rare with poor prognosis.
Limitations: Observational registry design — cannot establish causal explanations for survival changes.; Limited granularity of treatment data reported (only initial treatment categories: surgery, systemic therapy, radiotherapy).; Some subgroups (especially primary ovarian sarcomas) are small, limiting statistical power for trend tests.; Potential for registry coding/misclassification or missing data inherent to cancer registry sources..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
ReviewMechanismInconclusiveModerate evidenceTier 3 · early human
Klinicka onkologie : casopis Ceske a Slovenske onkologicke spolecnosti · Jul 2019
pleuropulmonary blastomamultinodular goiterovarian Sertoli-Leydig cell tumorcystic nephromamedulloepithelioma (ciliary body/iris)embryonal rhabdomyosarcoma (botryoid type)nasal epithelial/chondromesenchymal hamartomapituitary blastomapineoblastomadifferentiated thyroid carcinomapulmonary blastomawell-differentiated fetal lung adenocarcinomaanaplastic sarcoma of the kidneyprimary ovarian sarcomaPPB-like peritoneal sarcomamulticystic liver neoplasmsWilms tumor
This article summarizes the clinical features, genetic diagnosis, management, and surveillance recommendations for DICER1 syndrome, an inherited disorder caused by germline DICER1 pathogenic variants that predispose to a spectrum of benign and malignant tumors. It lists the most common associated tumors (e.g., pleuropulmonary blastoma, thyroid nodules, ovarian Sertoli-Leydig cell tumors) and gives recommended surveillance schedules and guidance on genetic testing and cascade testing for relatives. The authors note autosomal dominant inheritance with reduced penetrance and state diagnosis is by identification of a heterozygous germline DICER1 pathogenic variant.
Key findings
- DICER1 syndrome is caused by pathogenic variants in the DICER1 gene (located at chromosome 14q32.13) and is associated with increased risk of a spectrum of malignant and benign tumors.
- The most common clinical features include lung cysts and thyroid nodules; common neoplasms include pleuropulmonary blastoma, Sertoli-Leydig cell tumor, pediatric cystic nephroma, and differentiated thyroid carcinoma.
- A broad and variable tumor spectrum is reported, with many tumors occurring before age 40 and PPB typically presenting in children younger than seven years.
- Diagnosis is established by identification of a heterozygous germline DICER1 pathogenic variant presumed to cause loss of function.
- Management of DICER1-associated tumors is tumor-type dependent and may include surgery, chemotherapy, and in some cases radiation; surveillance recommendations (based on the 2016 International PPB Register) are provided for chest imaging, thyroid ultrasound, pelvic and abdominal ultrasound, ophthalmologic assessment, and neurologic monitoring.
- Genetic counseling is recommended, with cascade testing of at-risk first-degree relatives and consideration of testing soon after birth because screening often begins in infancy.
Limitations: This is a review/clinical overview rather than original primary quantitative research.; Surveillance recommendations are presented but the abstract does not provide quantitative evidence of their effectiveness.; Recommendations appear to be based on existing guidance (2016 International PPB Register) and may reflect expert consensus rather than prospective trial data.; Variable penetrance and broad tumor spectrum limit precise risk prediction for individual carriers..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Human · observationalMixed resultsLimited evidenceTier 3 · early humann = 24
Chinese medical journal · May 2011 · retrospective review
primary ovarian sarcomaovarian carcinosarcoma (MMMT)ovarian leiomyosarcomaovarian endometrial stromal sarcoma (ESS)
This retrospective review of 24 patients with primary ovarian sarcoma treated at a single center (1988–2007) analyzed clinicopathologic features and survival after surgery and chemotherapy. Optimal debulking was associated with longer median survival (28 months) compared with suboptimal debulking (6 months, P = 0.02). Most patients (23/24) received platinum-based chemotherapy (mean 6.6 ± 5.0 courses), but responses were described as unsatisfactory. The median survival for the whole cohort was 18.7 months, with 1-year and 2-year survival rates of 58% and 29%, respectively.
Reported effects: median survival (optimal debulking group) 28 mo · 1-year survival rate (optimal debulking group) 71% · +7 more
Studied with: surgery, chemotherapy, radiation.
Key findings
- Mean age was 54.3 ± 10.3 years; 16 patients were postmenopausal; abdominal pain occurred in 14 patients.
- Histology: 16 carcinosarcoma (MMMT), 2 leiomyosarcoma, 6 endometrial stromal sarcoma.
- Optimal debulking group: median survival 28 months; 1-year survival rate 71%.
- Suboptimal debulking group: median survival 6 months (P = 0.02); 1-year survival rate 29%.
- Median survival for the entire group was 18.7 months.
- One-year survival rate for entire group was 58%; two-year survival rate 29%.
- Twenty-three patients received chemotherapy (mostly platinum-based); three patients received chemoradiation.
- Mean number of courses of combined chemotherapy was 6.6 ± 5.0, and the response was described as unsatisfactory.
Limitations: Retrospective, single-center study design; Small sample size (n = 24); Heterogeneous histologic subtypes pooled together (MMMT, LS, ESS); Heterogeneous and incompletely specified chemotherapy regimens (most 'platinum-based' but not standardized); No randomized or controlled comparison; Limited reporting of response definitions and follow-up details.
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Animal studyReported positivePreclinical onlyTier 2 · animal
International journal of pharmaceutics · Jan 2010
M5076 ovarian sarcomaovarian neoplasm
This study examined how cucurbitacin E affects doxorubicin (DOX) handling in M5076 ovarian sarcoma. In vitro, cucurbitacin E suppressed DOX efflux from tumor cells in a manner consistent with involvement of multidrug resistance-associated protein (MRP) but not P-glycoprotein. In vivo, cucurbitacin E co-treatment increased DOX concentration in tumors shortly after administration while decreasing DOX in normal tissues.
Studied with: doxorubicin, MK-571.
Key findings
- Cucurbitacin E suppressed DOX efflux from M5076 ovarian sarcoma cells in vitro; MK-571 (an MRP inhibitor) also suppressed DOX efflux and the efficacy was the same for MK-571 and cucurbitacin E, implicating MRP in cucurbitacin E's effect.
- The effect of cucurbitacin E on DOX permeability did not appear to relate to P-glycoprotein (P-gp).
- In vivo, cucurbitacin E co-treatment significantly increased DOX concentration in the tumor within a short time after DOX administration and decreased DOX concentration in normal tissues.
- The authors speculate the different effects between tumor and normal tissues are related to differences in DOX transport systems on cell membranes.
- The authors state cucurbitacin E co-treatment was expected to increase DOX-induced antitumor activity without increasing DOX adverse reactions.
Limitations: Abstract provides no numeric data, sample sizes, species, dosing, timing, or statistical details.; Mechanistic links are inferred (speculation about MRP involvement and differences in transport systems) rather than conclusively proven in the abstract.; Findings are preclinical (in vitro and in vivo animal) and may not translate to humans.; No safety, toxicity, or therapeutic index data are provided in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Animal studyReported positivePreclinical onlyTier 2 · animal
Biochimica et biophysica acta · Dec 2003
In mice bearing M5076 ovarian sarcoma, co-administration of theanine increased tumor doxorubicin concentration, enhanced the antitumor activity of doxorubicin and suppressed hepatic metastasis, while not increasing doxorubicin levels in normal tissues or worsening markers of doxorubicin-induced toxicity. In vitro, theanine inhibited glutamate uptake and doxorubicin efflux from tumor cells, reduced intracellular glutamate, GSH and GS-DOX conjugate levels, and the authors propose involvement of MRP5/GS-X export; similar effects were seen with specific glutamate transporter inhibitors and with other chemotherapeutics.
Studied with: doxorubicin, other anthracyclines, cisplatin, irinotecan, green tea (oral).
Key findings
- In M5076 ovarian sarcoma-bearing mice, theanine significantly enhanced the inhibitory effect of DOX on tumor growth and increased the DOX concentration in the tumor, compared to DOX-alone group.
- Oral administration of theanine or green tea similarly enhanced the antitumor activity of DOX.
- The combination of theanine with DOX suppressed the hepatic metastasis of ovarian sarcoma.
- An increase in DOX concentration was not observed in normal tissues such as liver and heart, and theanine tended to normalize DOX-induced increases in lipid peroxide levels and reduction of glutathione peroxidase activity.
- In vitro, theanine inhibited the efflux of DOX from tumor cells and significantly inhibited glutamate uptake by M5076 cells similar to specific inhibitors.
- Two astrocytic high-affinity glutamate transporters, GLAST and GLT-1, were expressed in M5076 cells.
- Theanine-induced reduction of intracellular glutamate caused decreases in intracellular glutathione (GSH) and GS-DOX conjugate levels; expression of MRP5 suggests export of GS-DOX via the MRP5/GS-X pump, which theanine affected.
- DHK and L-serine-O-sulfate (SOS), specific glutamate transporter inhibitors, also enhanced DOX antitumor activity via inhibition of glutamate uptake.
- Theanine enhanced the antitumor activities of other anthracyclines, cisplatin and irinotecan in this experimental context.
Limitations: Preclinical study in a single mouse tumor model and in vitro cell line; no human data reported.; Abstract does not report sample sizes, dosing regimens, or duration of treatment.; Safety and efficacy in humans are not evaluated; applicability to clinical chemotherapy is speculative.; Mechanistic links are inferred from in vitro findings and expression data rather than directly proven in patients..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Lab · in vitroMechanismMixed resultsPreclinical onlyTier 1 · lab
Current drug metabolism · Dec 2001
Doxorubicinleukemiaovarian sarcoma (M5076)Ehrlich ascites carcinoma The paper examined how four anthracycline antibiotics (doxorubicin, pirarubicin, daunorubicin, idarubicin) are taken up by human cultured leukemia HL60 cells, human fresh mononuclear cells, and some mouse tumor cells. It reported that all four are incorporated via carrier-mediated transport but that the specific carriers differ between tumor and normal cells; a nucleoside transport system contributed to uptake of doxorubicin and pirarubicin in HL60 and some mouse tumor cells but not in mononuclear cells and not for daunorubicin or idarubicin. The authors propose that modifying an anthracycline to be a substrate for nucleoside transporters might allow more selective delivery to tumor cells.
Key findings
- Doxorubicin, pirarubicin, daunorubicin and idarubicin were incorporated via a common carrier-mediated system, but the carriers were different in HL60 tumor cells versus human fresh mononuclear cells.
- In HL60 cells a nucleoside transport system contributed at least in part to transport of doxorubicin and pirarubicin, but not to daunorubicin and idarubicin.
- The contribution of a nucleoside transport system to pirarubicin transport was also found in other tumor cells (mouse ovarian sarcoma M5076 and Ehrlich ascites carcinoma cells).
- In human mononuclear cells there was no involvement of a nucleoside transport system for the four anthracyclines examined.
- Authors suggest that modifying an anthracycline molecule to be a substrate for the nucleoside transport system could enable selective delivery to tumor cells.
Limitations: Experiments described are cell-based (in vitro) models—no in vivo or clinical data reported in the abstract.; Only a limited set of cell types were tested (HL60, human mononuclear cells, M5076, Ehrlich ascites), so generalizability is uncertain.; The therapeutic strategy proposed (modifying drugs for nucleoside transport) is speculative and not experimentally tested in vivo or clinically in this report..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Case reportInconclusiveLimited evidenceTier 3 · early humann = 1
American journal of clinical oncology · Jun 2000 · case report
granulocytic sarcoma of the ovaryovarian neoplasmacute myeloid leukemia (potentially related)
This is a case report of a 26-year-old woman who presented with a granulocytic sarcoma located in the ovary. The tumor occurred without any concurrent hematologic disorder according to the authors. No treatment details or follow-up outcomes are provided in the abstract.
Key findings
- Reported a primary granulocytic sarcoma arising in the ovary of a 26-year-old woman.
- The ovarian granulocytic sarcoma occurred in the absence of any hematologic disorder at the time of reporting.
Limitations: Single-patient case report — findings may not generalize.; Abstract provides no details on treatment, management, or follow-up outcomes.; Limited clinical and pathological detail in the abstract..
AI summary of the abstract, human-reviewed · Jun 2026. Describes what this study reported, not medical advice. View on PubMed
Animal studyReported positivePreclinical onlyTier 2 · animal
Cancer letters · Nov 1998
M5076 ovarian sarcoma
In mice bearing M5076 ovarian sarcoma, adriamycin alone did not inhibit tumor growth, while combining adriamycin with the green tea amino acid theanine reduced tumor weight to 62% of control. Theanine increased adriamycin concentration in tumors by 2.7-fold and decreased concentrations in normal tissues; in vitro theanine inhibited adriamycin efflux from tumor cells. Oral theanine or green tea also enhanced adriamycin's antitumor activity in this preclinical model.
Reported effects: tumor weight (% of control) 62% · intratumoral adriamycin concentration (fold change) 2.7
Studied with: adriamycin (doxorubicin).
Key findings
- Adriamycin alone did not inhibit tumor growth in M5076 tumor-bearing mice (qualitative).
- The combination of theanine and adriamycin significantly reduced the tumor weight to 62% of the control level.
- Theanine specifically increased the adriamycin concentration in the tumor by 2.7-fold.
- Theanine decreased adriamycin concentrations in normal tissues (qualitative).
- In vitro, theanine inhibited the efflux of adriamycin from tumor cells.
- Oral administration of theanine or green tea similarly enhanced the antitumor activity of adriamycin in this model.
Limitations: Preclinical study in mice and in vitro only—no human data.; Sample sizes and experimental doses are not reported in the abstract.; Single tumor model (M5076 ovarian sarcoma) evaluated.; No quantitative toxicity or safety data reported in the abstract.; Mechanistic conclusions rely in part on in vitro efflux data and may not be fully proven in vivo..
Preclinical (mouse and in vitro) evidence that theanine, a green tea component, can increase intratumoral doxorubicin levels and enhance antitumor activity against a mouse ovarian sarcoma model.
AI summary of the abstract, human-reviewed · Jul 2026. Describes what this study reported, not medical advice. View on PubMed