Overview
Rare ovarian cancer encompasses a heterogeneous group of uncommon malignant tumors arising from the ovary that do not fall into the category of common epithelial ovarian carcinomas (such as high-grade serous carcinoma). This broad category includes several distinct histological subtypes such as small cell carcinoma of the ovary (hypercalcemic type), ovarian clear cell carcinoma, low-grade serous carcinoma, mucinous ovarian carcinoma, granulosa cell tumors, Sertoli-Leydig cell tumors, ovarian carcinosarcoma, and other rare subtypes. Each subtype has distinct molecular, clinical, and prognostic features. These cancers primarily affect the female reproductive system but can spread to the peritoneum, lymph nodes, liver, lungs, and other organs. Symptoms of rare ovarian cancers are often nonspecific and may include abdominal or pelvic pain, bloating, early satiety, changes in bowel or bladder habits, unexplained weight loss, and an abdominal mass. Some subtypes present with specific features; for example, small cell carcinoma of the ovary (hypercalcemic type) often presents in young women with elevated serum calcium levels, while granulosa cell tumors may produce estrogen leading to abnormal uterine bleeding or precocious puberty in children. Diagnosis typically involves imaging, serum tumor markers, and histopathological examination of tissue obtained through surgery. Treatment approaches vary by subtype but generally include surgical debulking (cytoreductive surgery) as a cornerstone, often combined with platinum-based chemotherapy. Due to the rarity of these tumors, evidence-based treatment guidelines are limited, and management is frequently extrapolated from data on more common ovarian cancers or based on small case series and expert consensus. Emerging targeted therapies and immunotherapies are being investigated in clinical trials for specific subtypes. Prognosis varies widely depending on the histological subtype, stage at diagnosis, and response to treatment. Multidisciplinary care at specialized centers with experience in rare gynecological cancers is strongly recommended.
Also known as:
Multifactorial
Caused by a mix of several genes and environmental factors
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
7 eventsElahere: FDA approved
treatment of adult patients with folate receptor-alpha (FR?) positive, platinum-resistant epithelial ovarian, fallopian tube, or primary peritoneal cancer, who have received one to three prior systemic treatment regimens. Select patients for therapy based on an FDA-approved test.
Hoffmann-La Roche — PHASE2
Rubraca: FDA approved
For the maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in complete or partial response to platinum-based chemotherapy.
Lynparza: FDA approved
Maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube or primary peritoneal cancer, who are in a complete or partial response to platinum-based chemotherapy.
Zejula: FDA approved
Indicated for maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemotherapy
Gliadel: FDA approved
As an adjunct to surgery to prolong survival in patients with recurrent glioblastoma multiforme for whom surgical resection is indicated
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
7 availableOncoscint CR/OV
For determining the extent and location of extraphepatic malignant disease in patients with known ovarian cancer
Doxil
indicated for the treatment of patients with ovarian cancer whose disease has progressed or recurred after platinum-based chemotherapy
Elahere
treatment of adult patients with folate receptor-alpha (FR?) positive, platinum-resistant epithelial ovarian, fallopian tube, or primary peritoneal cancer, who have received one to three prior systemi…
treatment of adult patients with folate receptor-alpha (FR?) positive, platinum-resistant epithelial ovarian, fallopian tube, or primary peritoneal cancer, who have received one to three prior systemic treatment regimens. Select patients for therapy based on an FDA-approved test.
Rubraca
For the maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in complete or partial response to platinum-based chemotherapy.
Lynparza
Maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube or primary peritoneal cancer, who are in a complete or partial response to platinum-based chemotherapy.
Zejula
Indicated for maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemot…
Indicated for maintenance treatment of adult patients with recurrent epithelial ovarian, fallopian tube, or primary peritoneal cancer who are in a complete or partial response to platinum-based chemotherapy
Gliadel
As an adjunct to surgery to prolong survival in patients with recurrent glioblastoma multiforme for whom surgical resection is indicated
Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersWashington University School of Medicine
📍 St Louis, Missouri
👤 Meagan A Jacoby, M.D., Ph.D.
👤 Janssen Research & Development, LLC Clinical Trial
Stanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🔬 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
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🏥 NORDBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
Financial Resources
2 resourcesTravel Grants
No travel grants are currently matched to Rare ovarian cancer.
Community
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1 articlesCaregiver 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.
Common questions about Rare ovarian cancer
What is Rare ovarian cancer?
Rare ovarian cancer encompasses a heterogeneous group of uncommon malignant tumors arising from the ovary that do not fall into the category of common epithelial ovarian carcinomas (such as high-grade serous carcinoma). This broad category includes several distinct histological subtypes such as small cell carcinoma of the ovary (hypercalcemic type), ovarian clear cell carcinoma, low-grade serous carcinoma, mucinous ovarian carcinoma, granulosa cell tumors, Sertoli-Leydig cell tumors, ovarian carcinosarcoma, and other rare subtypes. Each subtype has distinct molecular, clinical, and prognostic
How is Rare ovarian cancer inherited?
Rare ovarian cancer follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
Are there clinical trials for Rare ovarian cancer?
Yes — 1 recruiting clinical trial is currently listed for Rare ovarian cancer on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Rare ovarian cancer?
9 specialists and care centers treating Rare ovarian cancer are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.
What treatment and support options exist for Rare ovarian cancer?
4 patient support programs are currently tracked on UniteRare for Rare ovarian cancer. See the treatments and support programs sections for copay assistance, eligibility, and contact details.