Overview
Malignant sex cord stromal tumor of the ovary is a rare type of ovarian cancer that starts in the cells that support and surround the eggs inside the ovary. These supporting cells — called sex cord and stromal cells — normally help produce hormones like estrogen and testosterone. When they become cancerous, they can grow into a tumor that may spread beyond the ovary. This group of tumors includes types such as granulosa cell tumors, Sertoli-Leydig cell tumors, and other less common subtypes. Together, they are sometimes called malignant ovarian sex cord stromal tumors or malignant SCSTs. Because these tumors often produce hormones, they can cause unusual symptoms related to hormone changes — such as irregular periods, early puberty in children, or signs of excess male hormones like facial hair. A lump or pain in the lower belly is also common. These hormone-related clues sometimes help doctors find the tumor earlier than other ovarian cancers. Treatment usually involves surgery to remove the tumor, often followed by chemotherapy. The outlook depends on how far the cancer has spread at diagnosis. Many patients, especially those caught at an early stage, do well with treatment. Ongoing monitoring after treatment is very important because these tumors can come back years later.
Key symptoms:
Pelvic or lower belly painA lump or swelling in the abdomenIrregular or abnormal menstrual periodsVaginal bleeding after menopauseBloating or feeling full quicklySigns of too much estrogen, such as breast tenderness or heavy periodsSigns of too much testosterone, such as facial hair growth, deepening voice, or acneEarly puberty in young girls (breast development, pubic hair before expected age)Nausea or vomitingUnintended weight lossFatigue or low energyDifficulty getting pregnant
Variable
Can be inherited in different ways depending on the underlying gene
Variable
Can begin at different ages, from infancy through adulthood
FDA & Trial Timeline
2 eventsBeihua Kong — PHASE3
Children's Hospitals and Clinics of Minnesota
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Malignant sex cord stromal tumor of ovary.
2 clinical trialsare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →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 Malignant sex cord stromal tumor of ovary.
Community
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Start the conversation →Latest news about Malignant sex cord stromal tumor of ovary
Disease timeline:
New recruiting trial: TC or BEP in Treating Patients With Ovarian Malignant Sex Cord-Stromal Tumors
A new clinical trial is recruiting patients for Malignant sex cord stromal tumor of ovary
Caregiver Resources
NORD Caregiver Resources
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Mental Health Support
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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 type of sex cord stromal tumor do I have, and what does that mean for my treatment and prognosis?,Should I have genetic testing for FOXL2 or DICER1, and should my family members be tested?,Is fertility-sparing surgery an option for me, and what are the risks?,What chemotherapy regimen do you recommend, and what side effects should I expect?,How will you monitor me for recurrence, and how often will I need follow-up visits and blood tests?,Are there any clinical trials I should consider?,What signs of recurrence should I watch for at home, and when should I call you right away?
Common questions about Malignant sex cord stromal tumor of ovary
What is Malignant sex cord stromal tumor of ovary?
Malignant sex cord stromal tumor of the ovary is a rare type of ovarian cancer that starts in the cells that support and surround the eggs inside the ovary. These supporting cells — called sex cord and stromal cells — normally help produce hormones like estrogen and testosterone. When they become cancerous, they can grow into a tumor that may spread beyond the ovary. This group of tumors includes types such as granulosa cell tumors, Sertoli-Leydig cell tumors, and other less common subtypes. Together, they are sometimes called malignant ovarian sex cord stromal tumors or malignant SCSTs. Beca
Are there clinical trials for Malignant sex cord stromal tumor of ovary?
Yes — 2 recruiting clinical trials are currently listed for Malignant sex cord stromal tumor of ovary on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Malignant sex cord stromal tumor of ovary?
5 specialists and care centers treating Malignant sex cord stromal tumor of ovary are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.