Overview
Endometrioid carcinoma of the ovary is a malignant epithelial tumor of the ovary that histologically resembles endometrial carcinoma of the uterus. It accounts for approximately 10-15% of all epithelial ovarian cancers. This cancer arises from the surface epithelium of the ovary or from foci of endometriosis (a condition where endometrial-like tissue grows outside the uterus), and there is a well-established association between ovarian endometriosis and the development of this tumor type. The disease primarily affects the female reproductive system, but as it progresses, it can spread to the peritoneum, lymph nodes, and distant organs. Key symptoms include pelvic or abdominal pain, abdominal bloating or distension, changes in bowel or bladder habits, early satiety, unexplained weight loss, and an adnexal mass detected on imaging. Many patients are diagnosed at an earlier stage compared to high-grade serous ovarian carcinoma, which contributes to a generally more favorable prognosis. Endometrioid ovarian carcinomas are frequently low-grade and may be associated with concurrent endometrial pathology, including synchronous endometrioid carcinoma of the uterus in up to 15-20% of cases. Treatment typically involves cytoreductive surgery (debulking), including total hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and staging procedures, followed by platinum-based combination chemotherapy (usually carboplatin and paclitaxel). Molecular profiling has identified frequent mutations in genes such as CTNNB1, PTEN, PIK3CA, ARID1A, and mismatch repair genes, with a subset of tumors demonstrating microsatellite instability. Patients with mismatch repair deficiency may be candidates for immune checkpoint inhibitor therapy. Hormonal therapy may also be considered in select cases. The association with Lynch syndrome (hereditary nonpolyposis colorectal cancer syndrome, caused by germline mutations in mismatch repair genes including MLH1, MSH2, MSH6, and PMS2) means that genetic counseling and testing should be offered, particularly in younger patients or those with a suggestive family history.
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
8 eventsTheolytics Limited — PHASE1
Swiss GO Trial Group — NA
Sidney Kimmel Comprehensive Cancer Center at Johns Hopkins — PHASE2
TORL Biotherapeutics, LLC — PHASE2
Theolytics Limited — PHASE1, PHASE2
Vall d'Hebron Institute of Oncology — PHASE2
Memorial Sloan Kettering Cancer Center
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Endometrioid carcinoma of ovary.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Endometrioid carcinoma of ovary at this time.
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Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Endometrioid carcinoma of ovary.
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Start the conversation →Latest news about Endometrioid carcinoma of ovary
Disease timeline:
New recruiting trial: Study of Senaparib in Combination With Temozolomide in ARID1A Mutation Associated Ovarian Cancer
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: Observational Study of Women With Endometrial Cancer Who Receive the Standard Treatment for Their Disease
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: A Phase 2 Study of Avutometinib (VS-6766) Plus Defactinib
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: Cirtuvivint/Olaparib in Breast Cancer Susceptibility Gene/Homologous Recombination Deficiency Platinum Resistant Ovarian Cancer
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: Trial of THEO-260 in Ovarian Cancer Patients
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: CATALINA-2: A Clinical Study of TORL-1-23 in Platinum-resistant Ovarian Cancer.
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: Status of HRD That Lead to a Benefit From Olaparib in Combination With Bevacizumab (STROBE Trial)
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: OV Precision: Study Examining the Benefit of a Tumor- and Patient-specific Cancer Therapy
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
New recruiting trial: Trial of THEO-260 (Administered Via Intraperitoneal Route) in Ovarian Cancer Patients
A new clinical trial is recruiting patients for Endometrioid carcinoma of ovary
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Common questions about Endometrioid carcinoma of ovary
What is Endometrioid carcinoma of ovary?
Endometrioid carcinoma of the ovary is a malignant epithelial tumor of the ovary that histologically resembles endometrial carcinoma of the uterus. It accounts for approximately 10-15% of all epithelial ovarian cancers. This cancer arises from the surface epithelium of the ovary or from foci of endometriosis (a condition where endometrial-like tissue grows outside the uterus), and there is a well-established association between ovarian endometriosis and the development of this tumor type. The disease primarily affects the female reproductive system, but as it progresses, it can spread to the p
How is Endometrioid carcinoma of ovary inherited?
Endometrioid carcinoma of ovary follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Endometrioid carcinoma of ovary typically begin?
Typical onset of Endometrioid carcinoma of ovary is adult. Age of onset can vary across affected individuals.
Which specialists treat Endometrioid carcinoma of ovary?
17 specialists and care centers treating Endometrioid carcinoma of ovary are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.