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Ovarian Sarcoma

A plain-English summary of the published research on Ovarian Sarcoma, reviewed and approved by our editors — not a hand-curated clinical overview.

Research summary · reviewed
Educational only: This page is not medical advice. Coordinate decisions with your oncology team.

Reviewed Sep 2026 · OncoForge editorial · How we review →

AI extractedhuman reviewedsources checkedretractions suppressed· last updated Sep 2026

Evidence at a glanceHuman · observationalMixed results⚠ Studies disagree
20 published studies that name Ovarian Sarcoma9 human studies approved & graded (trial, observational, or meta-analysis)3 human clinical studies in the Ovarian Sarcoma corpus112 source documents in the Ovarian Sarcoma corpus

last checked September 8, 2026

Why this grade?

Human · observational — Human observational evidence only — no trials.

Computed deterministically from the studies’ types and reported outcomes — not written by AI, and not a claim that anything works.

What the guidelines say

NCI PDQESMONCCNASCO

We link the authoritative guidelines rather than reproduce them. Below, the treatments on this page are split into standard care, guideline or regulatory options, supportive care, and studied but not standard so established care is not mixed with experimental or supportive items.

Guideline / FDA options - context-specific
  • doxorubicin
  • cisplatin
  • gemcitabine
Studied, not standard - investigational
  • green tea
  • cisplatin + doxorubicin
  • total abdominal hysterectomy + bilateral salpingo-oophorectomy
  • second-look laparotomy
  • cytarabine
  • DDA-LDOX(7.5)
  • DDA-LDOX
  • pirarubicin
  • platinum-based chemotherapy
  • vincristine + dactinomycin + Cyclophosphamide + radiotherapy
  • voreloxin
  • flagellin (TLR5 stimulation)
  • chemoradiotherapy
  • cholate + taurocholate
  • dihydrokainate (DHK)
  • L-serine-O-sulfate (SOS)
  • MK-571
  • theanine + doxorubicin
  • cytoreductive surgery + hyperthermic intraperitoneal chemotherapy
  • surgical cytoreduction
  • Bowman-Birk inhibitor (BBI)
  • 5,7-dihydroxy-6,4'-dimethoxyflavone
  • Bowman-Birk inhibitor concentrate (BBIC)
  • cucurbitacin E
  • pancratistatin
  • silver nanoparticles synthesized from Diospyros kaki (AgNPs)
  • theanine
  • tyrosine kinase inhibitors
  • cyclophosphamide
  • vincristine

Read the guidelines

Cancer-specific deep links aren’t curated yet — these search the authoritative sources for Ovarian Sarcoma.

Treatment map: Ovarian Sarcoma

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Standard care plus every compound studied in the literature (each cited) and graded by evidence, organized by clinical readiness. A category, not a verdict that anything works — confirm anything here with your oncology team.

35
Interventions
0
Standard of care
2
Tested in people
0
Lab / animal
28
Named in lit.
7
Classes
Standard of care (0) Guideline option (5) Tested in people (2) Lab / animal only (0) Named in the literature (28)

Tested in people, by trial phase: phase not reported ×2

Clinical evidence
Preclinical evidence
Standard of care
Guideline option
Tested in people
Lab / animal only
Named in the literature
Surgery & procedures
—
—
—
—
4
Chemotherapy
—
5
2
—
9
Targeted therapy
—
—
—
—
1
Immunotherapy
—
—
—
—
1
Repurposed drugs
—
—
—
—
1
Supplements & natural agents
—
—
—
—
7
Other
—
—
—
—
5

Columns group into clinical evidence (used in, or tested on, people) and preclinical evidence (lab/animal, or only named in the literature). Cell = number of interventions; a dashed cell means none recorded there.

Established care — detail (5)
Chemotherapy
doxorubicinAdjuvant (after surgery)
FDA-approved for this cancer.
—Guideline option
doxorubicin
FDA-approved for this cancer.
—Guideline option
cisplatinAdjuvant (after surgery)
FDA-approved for this cancer.
—Guideline option
gemcitabine
FDA-approved for this cancer.
—Guideline option
cisplatin
FDA-approved for this cancer.
—Guideline option
Investigational & adjunct compounds — detail (30)
Named in the literature
green teacisplatin + doxorubicintotal abdominal hysterectomy + bilateral salpingo-oophorectomysecond-look laparotomycytarabineDDA-LDOX(7.5)DDA-LDOXpirarubicinplatinum-based chemotherapyvincristine + dactinomycin + Cyclophosphamide + radiotherapy· Adjuvant (after surgery)voreloxinflagellin (TLR5 stimulation)chemoradiotherapycholate + taurocholatedihydrokainate (DHK)L-serine-O-sulfate (SOS)MK-571theanine + doxorubicincytoreductive surgery + hyperthermic intraperitoneal chemotherapysurgical cytoreductionBowman-Birk inhibitor (BBI)5,7-dihydroxy-6,4'-dimethoxyflavoneBowman-Birk inhibitor concentrate (BBIC)cucurbitacin Epancratistatinsilver nanoparticles synthesized from Diospyros kaki (AgNPs)theaninetyrosine kinase inhibitors

"Tested in people" rows show the highest trial phase found in that compound's cited human studies (Phase I–IV; "phase not reported" = a human study with no phase tag). "Studied" = named in the cited literature for this cancer. "FDA ✓" = FDA-approved for this cancer; "off-label" = an FDA-approved drug used outside its approved indications (per openFDA). Not a claim that anything works.

Reported figures

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Snapshot

The essentials in ~60 seconds — every line is drawn from the cited sources below.

What it is
Mixed mesodermal (sarcomatous) tumors of the ovary are rare; primary ovarian sarcomas are often very aggressive and commonly present with widespread extraovarian metastasis, with most patients reported at advanced (FIGO Stage III–IV) disease. [1][2]
Survival
Overall prognosis is poor: one series reported a median survival of 18.7 months for the whole group (one‑year survival 58%, two‑year 29%), while a different series found a median survival of 3 years among patients treated with combination cytotoxic chemotherapy. [3][1]
Standard treatment
Primary surgery aiming for optimal cytoreduction (residual ≤ 1 cm) is the goal—typically total abdominal hysterectomy with bilateral salpingo‑oophorectomy ± omentectomy—followed by systemic, usually platinum‑based, chemotherapy (in some series cisplatin + doxorubicin was used). [1][3][2][4]
Key test
Loss of SMARCA4 (BRG1) protein is used to diagnose SCCOHT/ovarian rhabdoid tumor, and germline DICER1 testing is used to identify DICER1 syndrome when suspected. [5][6]
Biggest challenge
The main clinical problem is aggressive, often widely metastatic disease at presentation that makes optimal surgical debulking difficult and contributes to poor outcomes. [1][2]

Ask about Ovarian Sarcoma

Answers come only from the cited sources on this page — with the supporting evidence shown. If the sources here don't cover your question, it will say so. Educational information, not medical advice.

Key numbers & factors

Survival by stage

StageSurvivalNotes
Stage IIsarcoma 90.5 months vs epithelial carcinoma 113 months (p=0.048) [7]
Stage IIIB62 months vs 81 months [7]
Stage IIIC14.5 months vs 55 months (p=0.007) [7]
Stage IV2 months vs 6 months (p=0.09) [7]

Risk factors

  • ▲ increases riskDICER1 pathogenic variant / DICER1 syndrome — majority of tumors in this syndrome occur in individuals younger than age 40 years [6]

Biomarkers

  • SMARCA4 (BRG1) loss · diagnostic marker for SCCOHT/ovarian rhabdoid tumor [5]
  • DICER1 mutation (germline)Actionablediagnosis of DICER1 syndrome (guides genetic diagnosis/management) [6]
  • CA 125 · can be elevated preoperatively and lower preop levels (<75 U/mL) associated with better survival in one series [2][8]
  • BCP-20 (FBXO39) · detected in ovary and sarcoma cell lines; proposed as a cancer/testis antigen for diagnosis or immunotherapy [9]
  • c-kit (CD117) · rarely expressed in mesenchymal ovarian tumors [10]
  • GLAST/GLT-1, MRP5, low topoisomerase II (in M5076 cells) · features of an experimental M5076 ovarian sarcoma model linked to lower anthracycline sensitivity [11][12]
  • Connexin 43 (Cx43) · increased relative density after BBI treatment in M5076-bearing mice (experimental) [13]

9 sections — tap any heading to expand its cited detail. Key points are above.

Overview7 points
  • Mixed mesodermal sarcoma of the ovary is a rare clinical entity. [1]
  • Primary ovarian sarcomas are often very aggressive tumors with widespread metastasis at the time of presentation, making optimal tumor debulking difficult. [1]
  • Primary ovarian endometrioid stromal sarcomas often have an indolent course with a better prognosis than other ovarian sarcomas. [14]
  • Ovarian small cell carcinoma of the hypercalcemic type (SCCOHT)/ovarian rhabdoid tumor is a rare and highly malignant tumor that typically occurs in young women. [5]
  • A retrospective series reported that preoperative CA 125 levels were elevated (> 35 U/mL) in 93% of patients with ovarian sarcomas. [2]
Show 2 lab & early-research findings
  • The M5076 ovarian sarcoma model has been studied in mice in multiple preclinical reports; M5076 cells have been used to investigate anthracycline transport and comparative cytotoxicity, and in vitro experiments indicated that theanine (an amino acid found in green tea leaves that has been previously indicated to enhance the antitumor activity of doxorubicin) increased the intracellular concentration of doxorubicin remaining in M5076 cells. [13][12][15]3 sources
  • Metastases to the breast from neoplasms originating in organs other than the breast are described as very rare; one reported case involved a 55-year-old woman with a granulosa cell tumor of the ovary with sarcomatous differentiation who later presented with several fast-growing nodular tumors in both breasts and imaging suggesting liver metastasis, and fine needle aspiration biopsy diagnosed metastatic sarcoma. [16]
Epidemiology6 points
  • The median age at presentation in the reported series was 67.5 years. [1]
  • Findings at laparotomy demonstrated extraovarian metastasis in 33/35 patients in the series. [1]
  • One hundred twelve patients (65%) with AGUS Pap smears favoring reactive changes were found to harbor 13 preinvasive and invasive cervical squamous neoplasias and 1 ovarian sarcoma (total, 12.5% of patients with smears). [17]
  • In DICER1 syndrome, the majority of tumors occur in individuals younger than age 40 years. [6]
  • In a retrospective series, patients with ovarian sarcoma had a mean age of 54.3 ± 10.3 years and 16 of 24 were postmenopausal. [3]
  • In one retrospective series of 47 women with primary ovarian sarcomas, 41 patients (87%) presented with International Federation of Gynecology and Obstetrics Stage III or IV disease. [2]
Standard management6 points
  • Sources report that most chemotherapy regimens were platinum-based, and although the optimal consolidation regimen remains unknown, platinum should be included as part of the regimen; systemic chemotherapy is often needed. [3][2][4]3 sources
  • Surgical cytoreduction (optimal debulking) to a residual tumor burden of ≤ 1 cm is associated with improved outcome and is considered the goal of primary surgery in ovarian sarcoma. [3][2]
  • Total abdominal hysterectomy and bilateral salpingo-oophorectomy with or without omentectomy was performed in most patients in this series. [1]
  • Follow-up adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29 of 36 patients in the series. [1]
  • Thirteen patients had a second-look laparotomy in the reported series; the authors reported that second-look surgery offers little helpful information on the management of these tumors. [1]
  • Ovarian tumors in the context of DICER1 syndrome are managed primarily with surgical resection and may also require chemotherapy depending on stage and histology, with radiation generally reserved for relapsed disease. [6]
Treatments & compounds studied35 treatments

Chemotherapy

  • doxorubicin · 3 findings
    • This PEG-modified liposomal doxorubicin formulation was reported to maintain a high doxorubicin concentration in a tumor for a long time. [18][19][12][15][20]5 sources
    • In Ehrlich ascites carcinoma tumor-bearing mice, oral administration of green tea enhanced 2.5-fold the inhibitory effects of doxorubicin on tumor growth. [21]
      fold increase in inhibitory effects with green tea 2.5 vs doxorubicin alone
      Source quote
      • “The oral administration of green tea enhanced 2.5-fold the inhibitory effects of doxorubicin on tumor growth.”
    • Adjuvant (after surgery)Doxorubicin was administered as part of adjuvant combination chemotherapy in the reported patient series. [1]
      count receiving adjuvant doxorubicin (within combination) 295-year survival 35%count undergoing primary surgical procedure 34
      Source quotes
      • “Follow-ups adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29/36 patients.”
      • “The combination of cisplatin and doxorubicin appears to have activity resulting in a survival of 35% at 5 years.”
      • “Total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy were performed in 34 patients,”
  • cisplatin + doxorubicin: The combination of cisplatin and doxorubicin was reported to result in 35% survival at 5 years. [1]
    count receiving adjuvant doxorubicin (within combination) 295-year survival 35%count undergoing primary surgical procedure 34
    Source quotes
    • “Follow-ups adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29/36 patients.”
    • “The combination of cisplatin and doxorubicin appears to have activity resulting in a survival of 35% at 5 years.”
    • “Total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy were performed in 34 patients,”
  • cytarabine: Addition of cytarabine decreased [3H]uridine uptake via CNT2, suggesting interaction with CNT2-mediated nucleoside uptake. [22]
  • gemcitabine: Addition of gemcitabine decreased [3H]uridine uptake via CNT2, suggesting interaction with CNT2-mediated nucleoside uptake. [22]
  • platinum-based chemotherapy: Platinum-based chemotherapy regimens were the most commonly used systemic therapy in one reviewed series (mean of 6.6 ± 5.0 courses) and, in a retrospective series of primary ovarian sarcomas, patients treated with platinum-based chemotherapy were reported to be significantly more likely to have a response and to have a survival advantage compared with those treated with other regimens. [3][2]
    mean number of courses of combined chemotherapy 6.6patients receiving chemoradiation 3
    Source quotes
    • “The mean number of courses of combined chemotherapy was 6.6 ± 5.0, and the response was unsatisfactory.”
    • “Among the patients, 23 patients received chemotherapy and most of regimens were based on platinum, 3 patients received chemoradiation.”
  • vincristine + dactinomycin + cyclophosphamide (VAC) with radiotherapy: Adjuvant (after surgery)Post-operative combination radiation therapy with vincristine, dactinomycin, and cyclophosphamide (VAC) was given and some patients achieved complete tumor control. [23]
Show 5 lab & early-research entries
  • cisplatin: Adjuvant (after surgery)Cisplatin was used as part of adjuvant combination chemotherapy in the reported patient series. [1]
    count receiving adjuvant doxorubicin (within combination) 295-year survival 35%count undergoing primary surgical procedure 34
    Source quotes
    • “Follow-ups adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29/36 patients.”
    • “The combination of cisplatin and doxorubicin appears to have activity resulting in a survival of 35% at 5 years.”
    • “Total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy were performed in 34 patients,”
  • DDA-LDOX(7.5): DDA-LDOX(7.5), a different double arms PEG-modified liposomal doxorubicin formulation, had a strong antitumor effect and maintained high doxorubicin concentration in tumors in mice bearing M5076 ovarian sarcoma cells. [18]
  • DDA-LDOX: Doxorubicin-loaded DDA-PEG-modified liposomes (DDA-LDOX) accumulated at sites of hepatic metastasis and, in a murine M5076 ovarian sarcoma hepatic metastasis model, the DDA-LDOX group had a survival ratio of 66.7% until 60 days after treatment. [24]
    survival ratio at 60 days 66.7% vs control / DOX solution / other PEG-modified liposomal DOX groups
    Source quote
    • “The survival time of M5076 ovarian sarcoma bearing mice in DDA-LDOX group was longer than those in control, DOX solution and the other PEG-modified liposomal DOX groups, and the survival ratio in DDALDOX group remained 66.7% until 60 days after treatment.”
  • pirarubicin (THP): Pirarubicin (THP) was reported to be taken up into M5076 cells in part via a Na+-dependent nucleoside transport system and was more cytotoxic to M5076 cells than doxorubicin. [25][12]
  • voreloxin: Voreloxin (formerly SNS-595) demonstrated broad anti-proliferative activity in vitro and strong dose-dependent tumor growth inhibition in solid tumor xenograft models including M5076 ovarian sarcoma, with tumor growth inhibition reported as 86% at 20 mg/kg weekly and 63-88% in 10 of 11 solid tumor xenograft models. [26]
    tumor growth inhibition 86%
    Source quote
    • “Administration of voreloxin at 20 mg/kg weekly for five doses effectively inhibited tumor growth (86%).”

Targeted therapy

  • tyrosine kinase inhibitors (e.g., STI-571) · tyrosine kinase inhibitors: Because mesenchymal tumors of the ovary rarely express c-kit, the report states it is unlikely that patients with these tumors will be candidates for currently available tyrosine kinase inhibitors. [10]

Immunotherapy

  • flagellin (TLR5 stimulation): Acute stimulation of TLR5 with flagellin was reported to boost anti-tumor immunity in established ovarian cancer models. [27]

Repurposed drugs

  • Bowman-Birk inhibitor (BBI): The Bowman-Birk protease inhibitor (BBI) was associated with increased Cx43 expression and negatively correlated with tumor weight and PCNA in M5076 ovarian sarcoma-bearing mice. [13][28]
    correlation r(2) between relative tumor weight and BBI dose 0.301, p <0.05correlation r(2) between PCNA and BBI dose 0.493, p <0.01correlation of relative tumor weight with BBI dose (r) 0.503, p <0.01correlation of Cx43 mRNA with BBI dose (r) 0.57, p <0.01
    Source quotes
    • “The relative tumor weight (p<0.05, r(2)=0.301) and proliferating cell nuclear antigen (PCNA, p<0.01, r(2)=0.493) were negatively correlated with the doses of BBI.”
    • “The relative tumor weight (p<0.05, r(2)=0.301) and proliferating cell nuclear antigen (PCNA, p<0.01, r(2)=0.493) were negatively correlated with the doses of BBI.”
    • “Relative tumor weight (p<0.01, r=0.503) was negatively correlated with the dose of BBI.”
    • “the relative density of Cx43 mRNA (p<0.01, r=0.570) ... was positively correlated with the dose of BBI”

Supplements & natural agents

  • green tea · 2 findings
    • Oral administration of green tea was reported to enhance the antitumor activity of doxorubicin in M5076-bearing mice. [11][20]
      tumor weight relative to control 62%, p significantly reduced vs controlincrease in tumor adriamycin concentration 2.7 vs adriamycin alone
      Source quotes
      • “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.”
    • The Doxorubicin concentration in the tumor was increased by the combination of green tea with doxorubicin, while the increase was not observed in normal tissues. [21]
      fold increase in inhibitory effects with green tea 2.5 vs doxorubicin alone
      Source quote
      • “The oral administration of green tea enhanced 2.5-fold the inhibitory effects of doxorubicin on tumor growth.”
  • cucurbitacin E: Cucurbitacin E co-treatment significantly increased doxorubicin concentration in the tumor and decreased doxorubicin concentration in normal tissues in M5076 ovarian sarcoma models, and when combined with doxorubicin was reported to decrease tumor size and tumor weight compared to doxorubicin alone. [29][19]
  • theanine: Theanine administration significantly enhanced the inhibitory effect of doxorubicin on tumor growth and increased doxorubicin concentration in M5076 tumors compared to doxorubicin alone. [11][15][20]3 sources
    tumor weight relative to control 62%, p significantly reduced vs controlincrease in tumor adriamycin concentration 2.7 vs adriamycin alone
    Source quotes
    • “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.”
Show 4 lab & early-research entries
  • 5,7-dihydroxy-6,4'-dimethoxyflavone: The flavone 5,7-dihydroxy-6,4'-dimethoxyflavone showed activity against seven human cancer cell lines, including NCI/ADR-RES ovary sarcoma, with an IC50 of 2.6 microg/mL. [30]
    IC50 2.6
    Source quote
    • “5,7-dihydroxy-6,4'-dimethoxyflavone isolated from this fraction was found to be active against seven human cancer cell lines, including NCI/ADR-RES ovary sarcoma, with an IC50 value of 2.6 microg/mL.”
  • Bowman-Birk inhibitor concentrate (BBIC): The Bowman-Birk inhibitor concentrate (BBIC) was reported to reduce proliferation associated with increased Cx43 expression in mice bearing M5076 ovarian sarcoma. [31]
    median number of macroscopic spontaneous metastases (pre-treated group) 1 vs 71.4 vs control groupmedian number of macroscopic spontaneous metastases (post-treated group) 1.9 vs 71.4 vs control group
    Source quotes
    • “The median numbers of macroscopic spontaneous metastases were significantly lower in the pre- (1.0±2.3) and post- (1.9±3.6) treated groups than in the control group (71.4±97.3).”
    • “The median numbers of macroscopic spontaneous metastases were significantly lower in the pre- (1.0±2.3) and post- (1.9±3.6) treated groups than in the control group (71.4±97.3).”
  • pancratistatin: Pancratistatin was reported to inhibit growth in vivo of murine M-5076 ovary sarcoma with life extension of 53-84% at 0.38-3.0 mg/kg. [32]
  • silver nanoparticles synthesized from Diospyros kaki (AgNPs): Silver nanoparticles synthesized from Diospyros kaki leaf extract were evaluated for cytotoxic effects in vitro including on the human ovarian sarcoma (Skov-3) cell line. [33]

Procedures & devices

  • total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy: Sources report that total abdominal hysterectomy and bilateral salpingo-oophorectomy with or without omentectomy were performed in 34 patients in this series. [1]
    count receiving adjuvant doxorubicin (within combination) 295-year survival 35%count undergoing primary surgical procedure 34
    Source quotes
    • “Follow-ups adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29/36 patients.”
    • “The combination of cisplatin and doxorubicin appears to have activity resulting in a survival of 35% at 5 years.”
    • “Total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy were performed in 34 patients,”
  • second-look laparotomy: Thirteen patients in the series underwent second-look laparotomy, which the authors report offers little helpful information for management. [1]
    count receiving adjuvant doxorubicin (within combination) 295-year survival 35%count undergoing primary surgical procedure 34
    Source quotes
    • “Follow-ups adjuvant chemotherapy consisting of cisplatin and doxorubicin was administered to 29/36 patients.”
    • “The combination of cisplatin and doxorubicin appears to have activity resulting in a survival of 35% at 5 years.”
    • “Total abdominal hysterectomy and bilateral salpingo-oophorectomy +/- omentectomy were performed in 34 patients,”
  • cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC): Cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS-HIPEC) for non-colorectal peritoneal surface malignancies, including sarcoma, is discussed but remains controversial due to lack of level 1 evidence. [34]
  • surgical cytoreduction: Surgical cytoreduction to a residual tumor burden of ≤ 1 cm was identified as the most significant prognostic factor in multivariate analysis of primary ovarian sarcomas. [2]

Other

  • chemoradiation · chemoradiotherapy: A small number of patients in the series received chemoradiation. [3]
  • dihydrokainate (DHK): Dihydrokainate (DHK), as a specific glutamate transporter inhibitor, enhanced the antitumor activity of doxorubicin via inhibition of glutamate uptake in M5076 models. [11]
  • L-serine-O-sulfate (SOS): L-serine-O-sulfate (SOS), a specific glutamate transporter inhibitor, enhanced the antitumor activity of doxorubicin via inhibition of glutamate uptake. [11]
  • MK-571: MK-571, a multidrug resistance associated protein (MRP) inhibitor, significantly suppressed doxorubicin efflux from M5076 ovarian sarcoma cells. [29]
  • theanine plus doxorubicin: Combination of theanine with doxorubicin significantly reduced the primary tumor in M5076 transplanted (s.c.) mice. [15]
Show 2 lab & early-research entries
  • broad-spectrum antibiotics (microbiota depletion): Depletion of intestinal bacteria with a cocktail of broad-spectrum antibiotics was reported to delay progression of tumor models of sarcoma and ovarian carcinoma in mice. [27]
  • cholate and taurocholate: Cotreatment with the bile acids cholate and taurocholate significantly increased Na(+)-dependent uptake of [3H]uridine and THP by M5076 cells and enhanced the cytotoxicity of THP and doxorubicin. [35]
Prognosis6 points
  • With aggressive treatment, patients with early stage disease can have long term survival; in follow-up of 21 patients with primary ovarian endometrioid stromal sarcoma, 10 were alive and free of disease from 4 to 21 years postoperatively, six were alive with disease from 1 to 22 years postoperatively, and five are known to have died of disease. [4][14]
  • In a reported series of primary ovarian sarcoma patients the median survival for the entire group was 18.7 months; the one-year survival rate for the cohort was 58% and the two-year survival rate was 29%; patients who underwent optimal debulking had a median survival of 28 months and a 1-year survival rate of 71%, while patients in the suboptimal debulking group had a median survival of 6 months (P = 0.02) and a 1-year survival rate of 29%. [3]
  • Survival and disease-free interval for patients with ovarian sarcoma are lower than those for patients with epithelial ovarian carcinoma; by FIGO stage, overall survival was: stage II — sarcoma 90.5 months vs epithelial carcinoma 113 months (p=0.048); stage IIIB — 62 months vs 81 months; stage IIIC — 14.5 months vs 55 months (p=0.007); stage IV — 2 months vs 6 months (p=0.09). [7]
  • Survival analysis in the reported series showed a median survival of 3 years among patients treated with combination cytotoxic chemotherapy. [1]
  • Patients with positive second-look findings, as well as all those who recurred clinically, subsequently died within 12 months despite trials with different second-line chemotherapeutic agents. [1]
Show 1 lab & early-research finding
  • Host NOS II expression was associated with fewer and smaller experimental lung metastases of M5076 cells in mice compared with NOS II-deficient hosts. [36]
Safety & interactions5 points
  • Cucurbitacin E co-treatment increased doxorubicin concentration in tumor while decreasing doxorubicin concentration in normal tissues and was expected to increase DOX-induced antitumor activity without an increase in adverse reactions. [29]
  • No statistically significant association with subsequent atherosclerotic cardiovascular disease (ASCVD) incidence was observed for ovarian cancer in the pooled cohort analyses. [37]
Show 3 lab & early-research findings
  • Theanine decreased adriamycin concentrations in normal tissues in the M5076 ovarian sarcoma model and, in vitro, inhibited the efflux of adriamycin from tumor cells; it did not increase indicators of doxorubicin-induced side toxicity and tended to normalize lipid peroxide levels and glutathione peroxidase activity. [20][11]
  • In a mouse model, the doxorubicin concentration in the tumor increased with green tea plus doxorubicin, while no increase was observed in normal tissues. [21]
  • In mice, oral green tea enhanced the inhibitory effects of doxorubicin on tumor growth by 2.5-fold. [21]
Key biomarkers7 points
  • CA 125 levels can be elevated in ovarian sarcoma; in one series preoperative CA 125 level < 75 U/mL was significantly associated with better survival. [2][8]
  • M5076 cells express the astrocytic high-affinity glutamate transporters GLAST and GLT-1 and the MRP5 protein, and have lower expression of topoisomerase II compared with Ehrlich cells, which was linked to lower anthracycline sensitivity. [11][12]
  • c-kit (CD117) is rarely expressed in mesenchymal tumors of the ovary. [10]
  • BBI treatment increased the relative density of connexin 43 (Cx43) in M5076-bearing mice. [13]
  • Loss of SMARCA4 (BRG1) protein expression is a useful diagnostic marker for SCCOHT/ovarian rhabdoid tumor. [5]
  • DICER1 syndrome is caused by mutations in the DICER1 gene and is diagnosed by identification of a heterozygous germline DICER1 pathogenic variant. [6]
Show 1 lab & early-research finding
  • The gene BCP-20 (FBXO39) was detected in various cancer cell lines including ovary and sarcoma and was suggested to be a new cancer/testis antigen that may be useful for diagnosis and immunotherapy. [9]
Biology & pathways6 points
  • CNT2 (concentrative nucleoside transporter 2) was cloned from mouse M5076 ovarian sarcoma cells and can mediate transport of cytidine and uridine via the same recognition site; cytidine analogues may be substrates for CNT2. [22]
  • Voreloxin's mechanism of action includes DNA intercalation and inhibition of topoisomerase II that causes selective DNA damage. [26]
  • The Bowman-Birk inhibitor (BBI) is suggested to induce negative growth control by increasing p27 accumulation through expression of connexin 43 as a gap junctional intercellular communication (GJIC)-dependent pathway. [28]
  • An unidentified Na(+)-dependent transport system expressed by M5076 cells is reported to contribute to the uptake and cytotoxicity of anthracyclines; pirarubicin (THP) uptake by M5076 cells depended on a Na+ gradient and was concluded to be partially taken up via a novel Na+-dependent transport system common to nucleosides, and cotreatment with both cholate and taurocholate significantly increased the Na(+)-dependent and cytidine-insensitive uptake of [3H]uridine and that of THP while the enhancement of THP uptake by the bile acids was reversed by cotreatment with 500 microM uridine. [35][25]
  • Commensal microorganisms are required for immune homeostasis at mucosal surfaces; depletion of intestinal bacteria with a cocktail of broad-spectrum antibiotics significantly delayed the progression of tumor models of sarcoma and ovarian carcinoma in mice; and acute stimulation of TLR5 with flagellin was reported to boost anti-tumor immunity in established ovarian cancer models. [27]
  • Host nitric oxide synthase II (NOS II) expression differentially modulated metastasis of M5076 ovarian sarcoma compared with a melanoma model; activated macrophages from NOS II+/+ mice produced NO-dependent cytotoxicity in sarcoma cells whereas macrophages from NOS II-/- mice did not. [36]
What we don't know yet4 points
  • Ovarian sarcomas are poorly studied; multicenter protocols and further research are needed to develop effective programs of treatment and to clarify management and outcomes. [7][4][2]3 sources
  • The role and optimal regimen of platinum-based chemotherapy in primary ovarian sarcoma remains unclear and requires further study. [3]
  • Because level 1 evidence is lacking, the role of cytoreductive surgery with hyperthermic intraperitoneal chemotherapy (CRS‑HIPEC) for non‑colorectal peritoneal surface malignancies remains uncertain; ongoing trials may help clarify its role. [34]
  • It has been proposed that modifying anthracyclines to be substrates for the nucleoside transport system could allow selective delivery to tumor cells. [38]

Sources

Every statement above is drawn from these reviewed sources. This page reports what they describe. Sources last checked September 8, 2026.

  1. Review articleMalignant mixed mesodermal ovarian tumor treatment and prognosis: a 20-year experience · 1997
  2. StudyPrimary ovarian sarcoma: analysis of prognostic variables and the role of surgical cytoreduction · 1998
  3. StudyPrimary sarcoma of the ovary: clinicopathological characteristics, prognostic factors and evaluation of therapy · 2011
  4. StudyOvarian sarcoma · 1987
  5. Clinical trialSMARCA4 (BRG1) loss of expression is a useful marker for the diagnosis of ovarian small cell carcinoma of the hypercalcemic type (ovarian rhabdoid tumor): a comprehensive analysis of 116 rare gynecologic tumors, 9 soft tissue tumors, and 9 melanomas · 2015
  6. StudyDICER1 Syndrome · 2019
  7. Clinical trialOvarian sarcoma carries a poorer prognosis than ovarian epithelial cancer throughout all FIGO stages: a single-center case-control matched study · 2014
  8. StudyThe clinical significance of pre-operative serum CA 125 in ovarian cancer · 1987
  9. Review articleIdentification of BCP-20 (FBXO39) as a cancer/testis antigen from colon cancer patients by SEREX · 2011
  10. Review articleLack of expression of c-kit protein (CD117) in mesenchymal tumors of the uterus and ovary · 2003
  11. Review articleTheanine and glutamate transporter inhibitors enhance the antitumor efficacy of chemotherapeutic agents · 2003
  12. Review articleRelationships between the in vitro cytotoxicity and transport characteristics of pirarubicin and doxorubicin in M5076 ovarian sarcoma cells, and comparison with those in Ehrlich ascites carcinoma cells · 2002
  13. Review articleRestoration of connexin 43 by Bowman-Birk protease inhibitor in M5076 bearing mice · 2005
  14. Review articlePrimary endometrioid stromal sarcoma of the ovary: a clinicopathologic study of 27 cases with morphologic and behavioral features similar to those of uterine low-grade endometrial stromal sarcoma · 2014
  15. Review articleCombination of theanine with doxorubicin inhibits hepatic metastasis of M5076 ovarian sarcoma · 1999
  16. Review articleFine needle aspiration biopsy diagnosis of metastatic neoplasms of the breast. A three-case report · 2005
  17. Review articlePapanicolaou smears of atypical glandular cells of undetermined significance: histological correlations and suggestions for management · 1999
  18. Clinical trialEnhanced antitumor activity of different double arms polyethyleneglycol-modified liposomal doxorubicin · 2013
  19. Review articleScreening of biochemical modulator by tumor cell permeability of doxorubicin · 2008
  20. StudyEnhancing effects of green tea components on the antitumor activity of adriamycin against M5076 ovarian sarcoma · 1998
  21. Review articleModulation of cancer chemotherapy by green tea · 1998
  22. Clinical trialCytidine is a novel substrate for wild-type concentrative nucleoside transporter 2 · 2006
  23. Clinical trialMixed mesodermal sarcoma of the ovary. Treatment with combination radiation therapy and chemotherapy · 1983
  24. Review articleEffect of Liposomes with Different Double Arms Polyethyleneglycol on Hepatic Metastasis Model Mice and Evaluation Using a Fluorescent Imaging Device · 2017
  25. Review articleUptake of the anthracycline pirarubicin into mouse M5076 ovarian sarcoma cells via a sodium-dependent nucleoside transport system · 2005
  26. Review articleVoreloxin, formerly SNS-595, has potent activity against a broad panel of cancer cell lines and in vivo tumor models · 2009
  27. Review articleSize does not matter: commensal microorganisms forge tumor-promoting inflammation and anti-tumor immunity · 2015
  28. Review articleConnexin 43-dependent tumor-suppressing effect of the Bowman-Birk protease inhibitor on M5076 ovarian sarcoma-bearing mice · 2008
  29. Clinical trialEnhancement of doxorubicin concentration in the M5076 ovarian sarcoma cells by cucurbitacin E co-treatment · 2010
  30. Review articleBioguided identification of antifungal and antiproliferative compounds from the Brazilian orchid Miltonia flavescens Lindl · 2014
  31. Review articleEffects of a single-dose administration of Bowman-Birk inhibitor concentrate on anti-proliferation and inhabitation of metastasis in M5076 ovarian sarcoma-bearing mice · 2008
  32. StudyAntineoplastic agents, 120. Pancratium littorale · 1986
  33. Review articleGreen synthesis of silver nanoparticles mediated Diospyros kaki L. (Persimmon): determination of chemical composition and evaluation of their antimicrobials and anticancer activities · 2023
  34. Review articleThe Role of Hyperthermic Intraperitoneal Chemotherapy for Non-colorectal Peritoneal Surface Malignancies · 2021
  35. Review articleContribution of an unidentified sodium-dependent nucleoside transport system to the uptake and cytotoxicity of anthracycline in mouse M5076 ovarian sarcoma cells · 2006
  36. Review articleInfluence of nitric oxide synthase II gene disruption on tumor growth and metastasis · 2000
  37. Review articleRisk of atherosclerotic cardiovascular disease after cancer diagnosis: findings from 3 prospective cohort studies · 2025
  38. Review articleContribution of specific transport systems to anthracycline transport in tumor and normal cells · 2001

What supports this page

The kinds of sources behind this page, strongest at the top. Faint rungs show what is not here yet.

Guideline
1
Meta-analysis
0
Systematic review
2
Randomized trial
0
Clinical trial
14
Observational
0
Case report
46
Review
41
Preclinical
0
Other
8

Living document — last change September 8, 2026: Cancer page updated. 3 recent updates logged.

Pooled evidence across studies

PubMed
  • OS: 74.7% (64–85 across studies) · (regimen unspecified)
    4 studies · 100% agree · consistent34602286
  • 5-year OS: 81% (29–88 across studies) · (regimen unspecified)
    3 studies · 67% agree · moderate · 1 flagged31326731
  • OS: 18.7 months (6–28 across studies) · platinum-based chemotherapy + surgery + chemotherapy + radiation
    3 studies · 33% agree · heterogeneous21740740
  • 1-year OS: 58% (29–71 across studies) · platinum-based chemotherapy + surgery + chemotherapy + radiation
    3 studies · 33% agree · heterogeneous21740740

Numbers pooled deterministically across 19 published studies that passed our figure-accuracy checks — a quick synthesis, not a formal meta-analysis. Updated 9/1/2026.

Compounds compared by evidence

PubMed

How to read this: Ranked by the strength and volume of the evidence — NOT by how well a treatment works. A higher rank means a compound has been studied more, or in stronger study designs (e.g. randomized trials over lab studies), not that it produces better outcomes. The effect column shows the largest pooled figure reported, not a head-to-head comparison.

#CompoundEvidence strengthStudiesLargest pooled effect
1Cyclophosphamide ChemotherapyHuman · observational1—
2Vincristine OtherHuman · observational1—
3Doxorubicin ChemotherapyAnimal only3—
4Cisplatin ChemotherapyAnimal only1—

Ranked across 4 compounds with published studies for this cancer. Updated 9/1/2026.

Medicines & supplements studied for Ovarian Sarcoma

PubMedFDAClinicalTrials.gov

Every drug, supplement, and other agent the published studies cover for Ovarian Sarcoma, ranked by how strong the evidence is — what studies report, not a recommendation. Tap any to see its full profile.

Medicines · 4

CyclophosphamideHuman · observationalMixed results1 human

Human observational evidence only — no trials.

Largest credible effect: complete tumor control - count 4, n=12 PMID 6616409 · effect sizes 1–20 across 6 studies

Most authoritative study: Mixed mesodermal sarcoma of the ovary. Treatment with combination radiation therapy and chemotherapy

Based on a single study.
ChemotherapyFDA off-label1 studyFull profile →
VincristineHuman · observationalMixed results1 human

Human observational evidence only — no trials.

Largest credible effect: complete tumor control - count 4, n=12 PMID 6616409 · effect sizes 1–20 across 6 studies

Most authoritative study: Mixed mesodermal sarcoma of the ovary. Treatment with combination radiation therapy and chemotherapy

Based on a single study.
ChemotherapyFDA off-label1 studyFull profile →
DoxorubicinAnimal onlyMixed results1 animal2 lab

Animal studies only — no human data.

Largest credible effect: age 62, n=1 PMID 36295661 · effect sizes 2–62 across 5 studies

Most authoritative study: Inhibition of glutamate transporter by theanine enhances the therapeutic efficacy of doxorubicin

No human studies yet · Findings conflict across studies · Effect sizes reported in only 1 of 4 studies.
ChemotherapyFDA approved4 studiesFull profile →
CisplatinAnimal onlyReported positive1 animal

Animal studies only — no human data.

Most authoritative study: Theanine and glutamate transporter inhibitors enhance the antitumor efficacy of chemotherapeutic agents

No human studies yet · No numeric effect sizes reported · Based on a single study.
ChemotherapyFDA approved1 studyFull profile →

What recent studies report in Ovarian Sarcoma

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.

20 studies9 human4 animal2 lab⚠ Conflicting evidenceMechanism (4)

Tracking 20 published studies of Ovarian Sarcoma: 9 in humans, 4 in animals, 2 in the lab, 5 reviews/other.

Reported direction across studies: 7 positive, 8 mixed, 2 negative, 3 inconclusive.

Findings conflict — both supportive and negative/mixed results exist (see below). Human evidence is limited.

These counts summarize what the studies reported; they are not a measure of whether anything works for Ovarian Sarcoma.

Compounds with studies mentioning Ovarian Sarcoma

Doxorubicin (4)Cisplatin (1)Vincristine (1)Cyclophosphamide (1)
Case reportMixed resultsLimited evidenceTier 3 · early humann = 1

Ovarian Sarcoma a Diagnostic Dilemma- A Case Report

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

High-Grade Endometrioid Stromal Sarcoma of the Ovary: Malignant Transformation of Ovarian Mature Cystic Teratoma

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

Incidence and treatment outcomes of ovarian sarcoma compared to epithelial ovarian cancer from the national cancer registry

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

Expanding the spectrum of dicer1-associated sarcomas

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

Overview of non-epithelial ovarian tumours: Incidence and survival in the Netherlands, 1989-2015

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

DICER1 Syndrome

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

Primary sarcoma of the ovary: clinicopathological characteristics, prognostic factors and evaluation of therapy

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

Enhancement of doxorubicin concentration in the M5076 ovarian sarcoma cells by cucurbitacin E co-treatment

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

Theanine and glutamate transporter inhibitors enhance the antitumor efficacy of chemotherapeutic agents

Biochimica et biophysica acta · Dec 2003

DoxorubicinCisplatinovarian sarcoma (M5076)

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

Contribution of specific transport systems to anthracycline transport in tumor and normal cells

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

Lab · in vitroReported positivePreclinical onlyTier 1 · lab

Inhibition of glutamate transporter by theanine enhances the therapeutic efficacy of doxorubicin

Toxicology letters · Apr 2001

Doxorubicinovarian sarcoma

This in vitro study in M5076 ovarian sarcoma cells found that theanine, an amino acid from green tea, inhibited glutamate uptake by acting on GLAST and GLT-1 transporters and reduced efflux of doxorubicin. The authors report that glutamate transport inhibitors had similar effects and conclude that inhibition of glutamate transport by theanine contributes to enhanced doxorubicin antitumor activity in these cells.

Studied with: doxorubicin.

Key findings
  • Theanine enhanced the antitumor activity of doxorubicin (DOX) due to inhibition of DOX efflux from tumor cells.
  • Glutamate transport inhibitors reduced the efflux of DOX, as well as theanine, from M5076 cells.
  • Theanine significantly inhibited glutamate uptake by M5076 cells in a concentration-dependent manner similar to specific inhibitors.
  • RT-PCR and Western blot analysis revealed the expression of GLAST and GLT-1 in M5076 cells.
  • Theanine competitively inhibits glutamate uptake by acting on GLAST and GLT-1, suggesting these transporters contribute to inhibition of DOX efflux.
Limitations: In vitro study only (single cell line, M5076); no animal or human data presented in the abstract.; Abstract reports no quantitative dosing or effect-size data.; Mechanistic link inferred from transporter inhibition and expression; genetic loss/gain experiments or in vivo confirmation not reported in the abstract..

AI summary of the abstract, human-reviewed · Sep 2026. Describes what this study reported, not medical advice. View on PubMed

Case reportInconclusiveLimited evidenceTier 3 · early humann = 1

Primary granulocytic sarcoma of the ovary

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

Browse all studies mentioning Ovarian Sarcoma →

Where the evidence is

What has been studied, and how strong it is, by topic. A dashed cell means no studies were found for that combination — a gap, not evidence of no effect. Open a row to see its studies.

CompoundHuman evidenceMechanismSafetyTrial
Cyclophosphamide1———
Vincristine1———
Doxorubicin—4——
Cisplatin—1——

Study mix

20 published studies by what they were done in. Lab and animal findings often do not carry over to people.

9 Human4 Animal2 Lab5 Review/other
Reported directionReported positive7Mixed results8Reported negative2Inconclusive3

Compounds with reported-positive results in Ovarian Sarcoma

Where at least one study reported a positive result, shown with the full picture, not just the wins. Positive results are more likely to be published, and most of these are early lab or animal studies that may not translate to people. This reports what studies found, not what works.

Preclinical only: lab / animal (2)
Doxorubicin2 positive2 negative/mixed1 animal2 lab
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.; In vitro study only (single cell line, M5076); no animal or human data presented in the abstract.; Abstract reports no quantitative dosing or effect-size data..
Cited positive studies (2)
Cisplatin1 positive1 animal
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..
Cited positive studies (1)

Evidence at a glance: compounds studied in Ovarian Sarcoma

A deterministic grade of what published studies report for each: strength of evidence, the reported direction, and the largest credible effect, strongest-evidence first. This summarizes findings; it is not a claim that anything works.

CyclophosphamideHuman · observationalMixed results1 human

Human observational evidence only — no trials.

Largest credible effect: complete tumor control - count 4, n=12 PMID 6616409 · effect sizes 1–20 across 6 studies

Most authoritative study: Mixed mesodermal sarcoma of the ovary. Treatment with combination radiation therapy and chemotherapy

Based on a single study.
VincristineHuman · observationalMixed results1 human

Human observational evidence only — no trials.

Largest credible effect: complete tumor control - count 4, n=12 PMID 6616409 · effect sizes 1–20 across 6 studies

Most authoritative study: Mixed mesodermal sarcoma of the ovary. Treatment with combination radiation therapy and chemotherapy

Based on a single study.
DoxorubicinAnimal onlyMixed results1 animal2 lab

Animal studies only — no human data.

Largest credible effect: age 62, n=1 PMID 36295661 · effect sizes 2–62 across 5 studies

Most authoritative study: Inhibition of glutamate transporter by theanine enhances the therapeutic efficacy of doxorubicin

No human studies yet · Findings conflict across studies · Effect sizes reported in only 1 of 4 studies.
CisplatinAnimal onlyReported positive1 animal

Animal studies only — no human data.

Most authoritative study: Theanine and glutamate transporter inhibitors enhance the antitumor efficacy of chemotherapeutic agents

No human studies yet · No numeric effect sizes reported · Based on a single study.

What the research shows for Ovarian Sarcoma

A plain-language summary of the reviewed studies OncoForge tracks for Ovarian Sarcoma. It reports what those studies described, not a claim that any compound or therapy helps or harms Ovarian Sarcoma. Most of this evidence is early, and findings often conflict.

  • A review of DICER1 syndrome summarized clinical features, genetic diagnosis, management, and surveillance recommendations and listed ovarian Sertoli–Leydig cell tumor among tumor types seen with germline DICER1 pathogenic variants.
  • An observational report described three pediatric sarcoma cases (including one ovarian tumor with a germline DICER1 mutation and two other sarcomas with somatic DICER1 mutations) and included a literature review of DICER1-associated sarcomas totaling about 83 cases.
  • Together, these publications emphasize an association between DICER1 genetic alterations and a subset of ovarian and other pediatric sarcomas, focusing on descriptive and biomarker/mechanistic findings rather than treatment effects.
  • The evidence consists mainly of case reports, small case series, and reviews; findings are preliminary and heterogeneous across reports.

Supportive & alternative options discussed

  • Exercise / prehabilitation: Also discussed as a supportive option for symptom management and quality-of-life in ovarian cancer care; these DICER1-focused studies did not evaluate exercise.
  • Mind–body (MBSR / CBT): Also discussed as a supportive option for coping, stress reduction, and quality-of-life in ovarian cancer care; these DICER1-focused studies did not evaluate mind–body approaches.
  • Acupuncture: Also discussed as a supportive option for symptom relief (e.g., pain, nausea) in ovarian cancer care; these DICER1-focused studies did not evaluate acupuncture.
  • Mistletoe (VAE): Also discussed by some patients and practitioners as a complementary option in cancer care; these DICER1-focused studies did not evaluate mistletoe.

What we don’t know yet

  • How common DICER1 mutations are across the full spectrum of ovarian sarcomas is not established.
  • Whether knowing DICER1 status alters clinical management or affects patient outcomes has not been demonstrated in these studies.
  • Optimal surveillance, long-term prognosis, and recurrence risk for DICER1-associated ovarian sarcomas remain unclear.
  • The mechanistic links between specific DICER1 alterations and tumor behavior, and whether they provide actionable therapeutic targets, are not fully defined.
Studies reporting DICER1 alterations in ovarian sarcomas are preliminary and based mainly on case reports, small series, and reviews, so they describe associations and biology but do not establish clinical benefit or guide treatment.

Clinical trials in Ovarian Sarcoma

33 ongoing · 89 completed · tracked from ClinicalTrials.gov. Recruiting is not the same as proven, and completed is not the same as positive — read the results. Not a recommendation.

Completed
23 stopped (terminated / withdrawn / suspended)

Search all trials on ClinicalTrials.gov →

Getting care & support

Nonprofit / Gov

Practical, vetted help for Ovarian Sarcoma — advocacy, paying for treatment, second opinions, and caregivers.

If you’re struggling emotionally, you don’t have to wait.

Advocacy & community

No dedicated organization for this specific cancer is curated yet — these general organizations can help in the meantime.

Financial help

  • PAN Foundation ↗ — Copay assistance funds by diagnosis (funds open and close as money allows). · status changes often — check the fund’s site
  • HealthWell Foundation ↗ — Copay and premium assistance funds by disease. · status changes often — check the fund’s site
  • CancerCare — financial assistance ↗ — Limited grants plus free financial counseling. · status changes often — check the fund’s site
  • Family Reach ↗ — Help with everyday living costs (rent, transport, food) during treatment. · status changes often — check the fund’s site
  • NeedyMeds ↗ — Searchable directory of drug patient-assistance and discount programs. · status changes often — check the fund’s site
What you’ll typically need to apply
  • Your diagnosis and, if you have it, the specific drug/treatment name (from your care team).
  • Insurance details — your member ID card, or a note that you're uninsured (some funds require active insurance, some don't).
  • Proof of income and household size (recent pay stubs, a tax return, or a benefits letter) — most funds are income-based.
  • Your prescriber's contact information; some programs need the clinic to submit part of the application.
  • Apply early and re-check: funds open and close as money is available, so a closed fund may reopen.

General guidance — each program sets its own eligibility. Confirm requirements on the program’s site.

Help paying for the medicines on this page

Second opinions

Caregiver support

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