Ovarian clear cell adenocarcinoma
diseaseOn this page
Also known as clear cell adenocarcinoma of ovaryovary clear cell adenocarcinoma
Summary
Ovarian clear cell adenocarcinoma (MONDO:0006045) is a disease with 2 GWAS associations across 1 studies and 39 clinical trials. Top therapeutic interventions include paclitaxel, topotecan, and cabozantinib. A subtype of ovarian clear cell cancer — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Prevalence: 1-9 / 1 000 000 (Europe) [Orphanet-validated]
- GWAS associations: 2
- Clinical trials: 39
Clinical features
Epidemiology
Prevalence records
24 prevalence record(s), Orphanet, top 20 (validated / broadest geography first):
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.32 | Europe | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.112 | Austria | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.361 | Belgium | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.201 | Bulgaria | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.147 | Croatia | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.165 | Czech Republic | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.24 | Estonia | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.359 | Finland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.182 | Germany | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.383 | Iceland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.395 | Ireland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.227 | Italy | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.162 | Latvia | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.166 | Lithuania | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.534 | Malta | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.425 | Norway | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.118 | Poland | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.119 | Portugal | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.273 | Slovakia | Validated |
| Annual incidence | 1-9 / 1 000 000 | 0.388 | Slovenia | Validated |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | ovarian clear cell adenocarcinoma |
| Mondo ID | MONDO:0006045 |
| EFO | EFO:1000042 |
| Orphanet | 398971 |
| DOID | DOID:5304 |
| ICD-11 | 315825558 |
| NCIT | C40078 |
| SNOMED CT | 763131005 |
| UMLS | C1518693 |
| MedGen | 276659 |
| GARD | 0021652 |
| Anatomy (UBERON) | UBERON:0000992 |
| Is cancer (heuristic) | no |
Also known as: clear cell adenocarcinoma of ovary · ovarian clear cell adenocarcinoma · ovary clear cell adenocarcinoma
Data availability: 2 GWAS associations (1 study) · 76 cell lines.
Disease family
This is a subtype of ovarian clear cell cancer. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › epithelial neoplasm › ovarian epithelial tumor › malignant epithelial tumor of ovary › ovarian clear cell cancer › ovarian clear cell adenocarcinoma
Related subtypes (1): ovarian clear cell malignant adenofibroma
Subtypes (1): ovarian clear cell cystadenocarcinoma
Genetics & variants
GWAS landscape
2 GWAS associations across 1 studies. Top hits map to 1 distinct genes (as reported by GWAS).
Top associations by p-value
| rsID | p-value | Gene | Risk allele | Odds ratio |
|---|---|---|---|---|
| rs11651755 | 7e-09 | HNF1B | T | 1.27 |
| rs7405776 | 2e-07 | HNF1B | A | 1.25 |
Top studies (by case count)
| Study | Lead author | Year | Cases | Controls | Title |
|---|---|---|---|---|---|
| GCST004417 | Phelan CM | 2017 | 1,366 | 40,941 | Identification of 12 new susceptibility loci for different histotypes of epithelial ovarian cancer. |
Variant details and genetic-evidence tiers
Tier distribution (top 50 variants)
| Tier | Variants |
|---|---|
| Tier 1: coding | 0 |
| Tier 2: splice/UTR | 0 |
| Tier 3: regulatory | 0 |
| Tier 4: intronic/intergenic | 2 |
MAF distribution
| Bucket | Variants |
|---|---|
| common (>=0.05) | 2 |
| low_freq (0.01-0.05) | 0 |
| rare (<0.01) | 0 |
| unknown | 0 |
Functional consequences
| Consequence | Count |
|---|---|
| intron_variant | 2 |
Top variants
| rsID | Chr | Pos | Alleles | MAF | Consequence | Gene | p-value | Tier |
|---|---|---|---|---|---|---|---|---|
| rs11651755 | 17 | 37739849 | T>C | 0.486 | intron_variant | HNF1B | 7e-09 | Tier 4: intronic/intergenic |
| rs7405776 | 17 | 37733029 | G>A,C,T | 0.412 | intron_variant | HNF1B | 2e-07 | Tier 4: intronic/intergenic |
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 39.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE2 | 14 |
| PHASE1 | 9 |
| PHASE3 | 7 |
| PHASE1/PHASE2 | 4 |
| PHASE2/PHASE3 | 2 |
| Not specified | 2 |
| EARLY_PHASE1 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT00565851 | PHASE3 | ACTIVE_NOT_RECRUITING | Carboplatin, Paclitaxel and Gemcitabine Hydrochloride With or Without Bevacizumab After Surgery in Treating Patients With Recurrent Ovarian, Epithelial, Primary Peritoneal, or Fallopian Tube Cancer |
| NCT02446600 | PHASE3 | ACTIVE_NOT_RECRUITING | Testing the Use of A Single Drug (Olaparib) or the Combination of Two Drugs (Cediranib and Olaparib) Compared to the Usual Chemotherapy for Women With Platinum Sensitive Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT02502266 | PHASE2/PHASE3 | ACTIVE_NOT_RECRUITING | Testing the Combination of Cediranib and Olaparib in Comparison to Each Drug Alone or Other Chemotherapy in Recurrent Platinum-Resistant Ovarian Cancer |
| NCT02839707 | PHASE2/PHASE3 | ACTIVE_NOT_RECRUITING | Pegylated Liposomal Doxorubicin Hydrochloride With Atezolizumab and/or Bevacizumab in Treating Patients With Recurrent Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT04575935 | PHASE3 | RECRUITING | Minimally Invasive Surgery After Neoadjuvant Chemotherapy for the Treatment of Stage IIIC-IV Ovarian, Primary Peritoneal, or Fallopian Tube Cancer, LANCE Trial |
| NCT00108745 | PHASE3 | UNKNOWN | Paclitaxel, Polyglutamate Paclitaxel, or Observation in Treating Patients With Stage III or Stage IV Ovarian Epithelial, Peritoneal Cancer, or Fallopian Tube Cancer |
| NCT00262847 | PHASE3 | COMPLETED | Carboplatin and Paclitaxel With or Without Bevacizumab in Treating Patients With Stage III or Stage IV Ovarian Epithelial, Primary Peritoneal, or Fallopian Tube Cancer |
| NCT00719303 | PHASE3 | UNKNOWN | Diet and Physical Activity Change or Usual Care in Improving Progression-Free Survival in Patients With Previously Treated Stage II, III, or IV Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT00951496 | PHASE3 | COMPLETED | Bevacizumab and Intravenous or Intraperitoneal Chemotherapy in Treating Patients With Stage II-III Ovarian Epithelial Cancer, Fallopian Tube Cancer, or Primary Peritoneal Cancer |
| NCT02068794 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | MV-NIS Infected Mesenchymal Stem Cells in Treating Recurrent Ovarian, Primary Peritoneal or Fallopian Tube Cancer |
| NCT03348631 | PHASE2 | ACTIVE_NOT_RECRUITING | Tazemetostat in Treating Patients With Recurrent Ovarian or Endometrial Cancer |
| NCT04739800 | PHASE2 | ACTIVE_NOT_RECRUITING | Comparison of Standard of Care Treatment With a Triplet Combination of Targeted Immunotherapeutic Agents |
| NCT04919629 | PHASE2 | RECRUITING | APL-2 and Pembrolizumab Versus APL-2, Pembrolizumab and Bevacizumab Versus Bevacizumab Alone for the Treatment of Recurrent Ovarian, Fallopian Tube, or Primary Peritoneal Cancer and Malignant Effusion |
| NCT05231122 | PHASE2 | RECRUITING | Pembrolizumab Combined With Bevacizumab With or Without Agonist Anti-CD40 CDX-1140 for the Treatment of Patients With Recurrent Ovarian Cancer |
| NCT05920798 | PHASE1/PHASE2 | RECRUITING | Vaccine Therapy Plus Pembrolizumab in Treating Advanced Ovarian, Fallopian Tube, or Primary Peritoneal Cavity Cancer |
| NCT06639074 | PHASE2 | RECRUITING | Folate Receptor Alpha Dendritic Cells (FRαDCs) or Placebo for the Treatment of Patients With Stage III or IV Ovarian, Fallopian Tube, or Primary Peritoneal Cancer, FAROUT Trial |
| NCT06730347 | PHASE2 | RECRUITING | A Study of Lorigerlimab in Participants With Advanced Solid Tumors |
| NCT00888615 | PHASE2 | COMPLETED | Elesclomol Sodium and Paclitaxel in Treating Patients With Recurrent or Persistent Ovarian Epithelial Cancer, Fallopian Tube Cancer, or Primary Peritoneal Cancer |
| NCT00979992 | PHASE2 | COMPLETED | Sunitinib Malate in Treating Patients With Persistent or Recurrent Clear Cell Ovarian Cancer |
| NCT01097746 | PHASE2 | COMPLETED | First-Line Treatment of Bevacizumab, Carboplatin, and Paclitaxel in Treating Participants With Stage III-IV Ovarian, Primary Peritoneal, and Fallopian Tube Cancer |
| NCT02315430 | PHASE2 | COMPLETED | Cabozantinib-S-Malate in Treating Patients With Recurrent or Progressive Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT02364713 | PHASE2 | TERMINATED | MV-NIS or Investigator’s Choice Chemotherapy in Treating Patients With Ovarian, Fallopian, or Peritoneal Cancer |
| NCT02713386 | PHASE1/PHASE2 | COMPLETED | Ruxolitinib Phosphate, Paclitaxel, and Carboplatin in Treating Patients With Stage III-IV Epithelial Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT02853318 | PHASE2 | COMPLETED | Pembrolizumab, Bevacizumab, and Cyclophosphamide in Treating Patients With Recurrent Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT02923739 | PHASE2 | TERMINATED | Paclitaxel and Bevacizumab With or Without Emactuzumab in Treating Patients With Platinum-Resistant Ovarian, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT03648489 | PHASE2 | COMPLETED | Dual mTorc Inhibition in advanCed/Recurrent Epithelial Ovarian, Fallopian Tube or Primary Peritoneal Cancer (of Clear Cell, Endometrioid and High Grade Serous Type, and Carcinosarcoma) |
| NCT06393751 | PHASE1/PHASE2 | WITHDRAWN | Testing the Addition of ASTX660 (Tolinapant) to the Usual Chemotherapy Treatment (Paclitaxel With or Without Bevacizumab) in Patients With Recurrent Ovarian Cancer |
| NCT04092270 | PHASE1 | ACTIVE_NOT_RECRUITING | A Study Combining the Peposertib (M3814) Pill With Standard Chemotherapy in Patients With Ovarian Cancer With an Expansion in High Grade Serous Ovarian Cancer and Low Grade Serous Ovarian Cancer |
| NCT05039801 | PHASE1 | RECRUITING | IACS-6274 With or Without Bevacizumab and Paclitaxel for the Treatment of Advanced Solid Tumors |
| NCT05226507 | PHASE1 | ACTIVE_NOT_RECRUITING | A Phase 1 Clinical Study of NXP800 in Subjects With Advanced Cancers and Expansion in Subjects With Ovarian Cancer |
| NCT05950464 | PHASE1 | RECRUITING | Testing Different Amounts of the Combination of Drugs M1774 and ZEN-3694 for the Treatment of Recurrent Ovarian and Endometrial Cancer |
| NCT00989651 | PHASE1 | COMPLETED | Carboplatin, Paclitaxel, Bevacizumab, and Veliparib in Treating Patients With Newly Diagnosed Stage II-IV Ovarian Epithelial, Fallopian Tube, or Primary Peritoneal Cancer |
| NCT01074411 | PHASE1 | COMPLETED | Intraperitoneal Bortezomib and Carboplatin in Treating Patients With Persistent or Recurrent Ovarian Epithelial Cancer, Fallopian Tube Cancer, or Primary Peritoneal Cancer |
| NCT02020707 | PHASE1 | COMPLETED | Nab-Paclitaxel and Bevacizumab in Treating Patients With Unresectable Stage IV Melanoma or Gynecological Cancers |
| NCT03924245 | PHASE1 | TERMINATED | Olaparib and Entinostat in Patients With Recurrent, Platinum-Refractory, Resistant Ovarian, Primary Peritoneal, Fallopian Tube Cancers |
| NCT05498597 | PHASE1 | UNKNOWN | AMT-151 in Patients With Selected Advanced Solid Tumours |
| NCT01504126 | EARLY_PHASE1 | COMPLETED | Propranolol Hydrochloride and Chemotherapy in Treating Patients With Ovarian, Primary Peritoneal, or Fallopian Tube Cancer |
| NCT07168083 | Not specified | NOT_YET_RECRUITING | Efficacy and Safety of Sacituzumab in Patients With OCCC After Immunotherapy Progression |
| NCT03641287 | Not specified | TERMINATED | The Effects of Exercise on Distress, Quality of Life, and Biomarkers in Ovarian Cancer Survivors |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| PACLITAXEL | 4 | 16 |
| TOPOTECAN | 4 | 4 |
| CABOZANTINIB | 4 | 3 |
| GEMCITABINE | 4 | 2 |
| OLAPARIB | 4 | 2 |
| ATEZOLIZUMAB | 4 | 1 |
| BEVACIZUMAB | 4 | 1 |
| CAPIVASERTIB | 4 | 1 |
| DIPHTHERIA TOXOID | 4 | 1 |
| PEGCETACOPLAN | 4 | 1 |
| RUXOLITINIB | 4 | 1 |
| SUNITINIB MALATE | 4 | 1 |
| TAZEMETOSTAT | 4 | 1 |
| TETANUS TOXOID | 4 | 1 |
| CEDIRANIB | 3 | 5 |
| VELIPARIB | 3 | 2 |
| ELESCLOMOL | 3 | 1 |
| EMACTUZUMAB | 3 | 1 |
| ENTINOSTAT | 3 | 1 |
| PACLITAXEL POLIGLUMEX | 3 | 1 |
| LORIGERLIMAB | 2 | 1 |
| TUVUSERTIB | 2 | 1 |
| ZEN-3694 | 2 | 1 |
| ASTX-660 | 1 | 1 |
| NEDISERTIB | 1 | 1 |
| CHEMBL5412235 | 0 | 1 |
| CHEMBL3109278 | 0 | 1 |
| CHEMBL5398431 | 0 | 1 |
| S-ROLIPRAM | 0 | 1 |