Clear cell adenocarcinoma of the ovary

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8Active trials20Specialists8Treatment centers1Financial resources

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Overview

Clear cell adenocarcinoma of the ovary (also called ovarian clear cell carcinoma or OCCC) is a rare subtype of epithelial ovarian cancer. It gets its name from the way the cancer cells look under a microscope — they appear clear or "hobnail-shaped" because of their high glycogen content. This cancer develops in the tissue that covers the outside of the ovary or lines cysts within the ovary. It is often associated with endometriosis, a condition where tissue similar to the uterine lining grows outside the uterus. Symptoms can include pelvic or abdominal pain, bloating, feeling full quickly when eating, changes in urination, and sometimes abnormal vaginal bleeding. Many women are diagnosed at an earlier stage compared to other ovarian cancers because the tumor can grow large and cause noticeable symptoms sooner. However, clear cell adenocarcinoma is known for being more resistant to standard platinum-based chemotherapy than other types of ovarian cancer. Treatment typically involves surgery to remove the tumor, often including removal of the ovaries, fallopian tubes, and uterus, followed by chemotherapy. Because this subtype does not respond as well to traditional chemotherapy, researchers are actively studying new targeted therapies and immunotherapy approaches. Early detection and treatment by a gynecologic oncologist experienced with this rare subtype are important for the best possible outcomes.

Also known as:

Key symptoms:

Pelvic or lower abdominal painAbdominal bloating or swellingFeeling full quickly when eatingFrequent or urgent need to urinateUnexplained weight loss or weight gainAbnormal vaginal bleedingFatigue and low energyBack painConstipation or changes in bowel habitsA noticeable mass or lump in the pelvisPain during intercourseBlood clots in the legs (deep vein thrombosis)High calcium levels in the blood (hypercalcemia)

Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

FDA & Trial Timeline

10 events
Jan 2026Liposomal Irinotecan Combined With Sintilimab and Anlotinib in the Treatment of Recurrent or Persistent Ovarian Clear Cell Carcinoma

Sun Yat-Sen Memorial Hospital of Sun Yat-Sen University — PHASE2

TrialNOT YET RECRUITING
Jun 2025INVIGORATE: A Study of QL1706 and Bevacizumab in Advanced First-Line Ovarian Clear Cell Carcinoma

Tongji Hospital — PHASE3

TrialNOT YET RECRUITING
Dec 2024Belzutifan in Recurrent Clear Cell Carcinoma of Gynecologic Origin

Dana-Farber Cancer Institute — PHASE2

TrialRECRUITING
Nov 2023Safety and Efficacy of Targeting PP2A in Ovarian Clear Cell Carcinoma Using Dostarlimab and LB-100

M.D. Anderson Cancer Center — PHASE1, PHASE2

TrialRECRUITING
Mar 2023A Comparative Study of the Treatment of Real-world Patients With Clear Cell Carcinoma of the Ovary

Tongji Hospital

TrialRECRUITING
Sep 2022Pembrolizumab and Lenvatinib in Clear Cell Ovarian Cancer

Elizabeth K. Lee MD — PHASE2

TrialACTIVE NOT RECRUITING
Jul 2022A Study of XmAb20717 (Vudalimab)in Patients With Selected Advanced Gynecologic and Genitourinary Malignancies

Xencor, Inc. — PHASE2

TrialACTIVE NOT RECRUITING
Dec 2021A Phase 1 Clinical Study of NXP800 in Subjects With Advanced Cancers and Expansion in Subjects With Ovarian Cancer

Nuvectis Pharma, Inc. — PHASE1

TrialACTIVE NOT RECRUITING
Sep 2019A Study of Tulmimetostat DZR123 (CPI-0209) in Patients With Advanced Solid Tumors and Lymphomas

Novartis Pharmaceuticals — PHASE1, PHASE2

TrialRECRUITING
May 2019Tazemetostat in Treating Patients With Recurrent Ovarian or Endometrial Cancer

National Cancer Institute (NCI) — PHASE2

TrialACTIVE NOT RECRUITING

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Treatments

No FDA-approved treatments are currently listed for Clear cell adenocarcinoma of the ovary.

8 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.

View clinical trials →

Clinical Trials

8 recruitingView all trials with filters →
Phase 24 trials
Belzutifan in Recurrent Clear Cell Carcinoma of Gynecologic Origin
Phase 2
Actively Recruiting
PI: Panagiotis Konstantinopoulos, MD, PhD (Dana-Farber Cancer Institute) · Sites: Boston, Massachusetts; Boston, Massachusetts · Age: 1899 yrs
Tazemetostat in Treating Patients With Recurrent Ovarian or Endometrial Cancer
Phase 2
Active
PI: Ramez N Eskander (NRG Oncology) · Sites: Anchorage, Alaska; Anchorage, Alaska +582 more · Age: 1899 yrs
A Study of XmAb20717 (Vudalimab)in Patients With Selected Advanced Gynecologic and Genitourinary Malignancies
Phase 2
Active
PI: Jolene Shorr (Xencor, Inc.) · Sites: Prescott, Arizona; La Jolla, California +15 more · Age: 1899 yrs
Pembrolizumab and Lenvatinib in Clear Cell Ovarian Cancer
Phase 2
Active
PI: Elizabeth K Lee, MD (Dana-Farber Cancer Institute) · Sites: Chicago, Illinois; Boston, Massachusetts +1 more · Age: 1899 yrs
Phase 11 trial
A Phase 1 Clinical Study of NXP800 in Subjects With Advanced Cancers and Expansion in Subjects With Ovarian Cancer
Phase 1
Active
PI: Udai Banerji, Prof (Institute of Cancer Research, Royal Marsden Founda) · Sites: Phoenix, Arizona; La Jolla, California +20 more · Age: 1899 yrs
Other1 trial
A Comparative Study of the Treatment of Real-world Patients With Clear Cell Carcinoma of the Ovary
Actively Recruiting
· Sites: Wuhan, Hubei · Age: 1875 yrs

Specialists

20 foundView all specialists →
RE
Ramez N Eskander
ENCINITAS, CA
Specialist
PI on 2 active trials
JF
John H Farley
Specialist
PI on 2 active trials2 Clear cell adenocarcinoma of the ovary publications
SP
Sandip P Patel
Birmingham, Alabama
Specialist

Rare Disease Specialist

PI on 1 active trial
PP
Panagiotis Konstantinopoulos, MD, PhD
BOSTON, MA
Specialist
PI on 2 active trials
KP
Kan Yonemori, MD, PhD
Specialist
PI on 2 active trials
HL
Huaiwu Lu
Specialist
PI on 2 active trials129 Clear cell adenocarcinoma of the ovary publications
LG
Lilian T Gien
Specialist
PI on 2 active trials1 Clear cell adenocarcinoma of the ovary publication
EM
Elizabeth K Lee, MD
Specialist
PI on 1 active trial
JF
John Farley
Specialist
PI on 1 active trial2 Clear cell adenocarcinoma of the ovary publications
RG
Rosalind Glasspool
Specialist
PI on 1 active trial
CH
Chih-Ming Ho
Specialist
PI on 1 active trial
HG
Hongyan Guo
Specialist
PI on 1 active trial
LX
Libing Xiang
Specialist
PI on 2 active trials
LC
Larry J Copeland
HILLIARD, OH
Specialist
PI on 1 active trial
CL
Charles N Landen
CHARLOTTESVILLE, VA
Specialist
PI on 1 active trial
JS
Jolene Shorr
Specialist
PI on 1 active trial
AD
Amir Jazaeri, M D
Specialist
PI on 1 active trial

Treatment Centers

8 centers
🏥 NORD

Baylor College of Medicine Rare Disease Center

Baylor College of Medicine

📍 Houston, TX

🏥 NORD

Stanford Medicine Rare Disease Center

Stanford Medicine

📍 Stanford, CA

🔬 UDN

NIH Clinical Center Undiagnosed Diseases Program

National Institutes of Health

📍 Bethesda, MD

🔬 UDN

UCLA UDN Clinical Site

UCLA Health

📍 Los Angeles, CA

🔬 UDN

Baylor College of Medicine UDN Clinical Site

Baylor College of Medicine

📍 Houston, TX

🔬 UDN

Harvard/MGH UDN Clinical Site

Massachusetts General Hospital

📍 Boston, MA

🏥 NORD

Mayo Clinic Center for Individualized Medicine

Mayo Clinic

📍 Rochester, MN

👤 Mayo Clinic Center for Individualized Medicine

🏥 NORD

UCLA Rare Disease Day Program

UCLA Health

📍 Los Angeles, CA

Financial Resources

1 resources

Hexalen

Medimmune Oncology, Inc.

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copay card
copay assistancePatient Assistance
Accepting applications

Travel Grants

No travel grants are currently matched to Clear cell adenocarcinoma of the ovary.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Clear cell adenocarcinoma of the ovary

1 articles
ResearchPUBMEDMar 26, 2026
Primary Clear Cell Adenocarcinoma of the Female Urethra: A Case Report of a Rare Disease.
Doctors found a very rare type of cancer in a woman's urethra (the tube that carries urine out of the body). This cancer, called clear cell adenocarcinoma, is s
See all news about Clear cell adenocarcinoma of the ovary

Caregiver Resources

NORD Caregiver Resources

Support, advocacy, and financial assistance for caregivers of rare disease patients.

Mental Health Support

Rare disease caregiving can be isolating. Connect with counseling and peer support.

Family & Caregiver Grants

Financial assistance programs specifically for caregivers of rare disease patients.

Social Security Disability

Learn how rare disease patients may qualify for SSDI/SSI benefits.

Questions for your doctor

Bring these to your next appointment

  • Q1.What stage is my cancer, and has it spread beyond the ovary?,Was the surgeon able to remove all visible cancer during my operation?,Given that clear cell carcinoma can be resistant to standard chemotherapy, what treatment plan do you recommend for me specifically?,Has my tumor been tested for molecular markers like ARID1A, PIK3CA, or microsatellite instability that might guide targeted therapy?,Should I have genetic testing for hereditary cancer syndromes like BRCA mutations or Lynch syndrome?,Are there any clinical trials available for clear cell ovarian cancer that I might be eligible for?,What is my expected prognosis, and what follow-up monitoring schedule will I need?

Common questions about Clear cell adenocarcinoma of the ovary

What is Clear cell adenocarcinoma of the ovary?

Clear cell adenocarcinoma of the ovary (also called ovarian clear cell carcinoma or OCCC) is a rare subtype of epithelial ovarian cancer. It gets its name from the way the cancer cells look under a microscope — they appear clear or "hobnail-shaped" because of their high glycogen content. This cancer develops in the tissue that covers the outside of the ovary or lines cysts within the ovary. It is often associated with endometriosis, a condition where tissue similar to the uterine lining grows outside the uterus. Symptoms can include pelvic or abdominal pain, bloating, feeling full quickly whe

How is Clear cell adenocarcinoma of the ovary inherited?

Clear cell adenocarcinoma of the ovary follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Clear cell adenocarcinoma of the ovary typically begin?

Typical onset of Clear cell adenocarcinoma of the ovary is adult. Age of onset can vary across affected individuals.

Are there clinical trials for Clear cell adenocarcinoma of the ovary?

Yes — 8 recruiting clinical trials are currently listed for Clear cell adenocarcinoma of the ovary on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.

Which specialists treat Clear cell adenocarcinoma of the ovary?

20 specialists and care centers treating Clear cell adenocarcinoma of the ovary are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Clear cell adenocarcinoma of the ovary?

1 patient support program are currently tracked on UniteRare for Clear cell adenocarcinoma of the ovary. See the treatments and support programs sections for copay assistance, eligibility, and contact details.