Overview
Malignant non-dysgerminomatous germ cell tumors of the ovary are a group of rare, aggressive ovarian cancers that arise from primordial germ cells. This category encompasses several histological subtypes including yolk sac tumor (endodermal sinus tumor), immature teratoma, embryonal carcinoma, choriocarcinoma, and mixed germ cell tumors. These tumors are distinct from dysgerminomas and tend to occur predominantly in children, adolescents, and young women, typically during the reproductive years. These tumors primarily affect the ovary but can spread to other structures within the pelvis and abdomen, and may metastasize to the lungs, liver, and lymph nodes. Key symptoms include abdominal or pelvic pain, abdominal distension or a palpable mass, and in some cases menstrual irregularities or precocious puberty in younger patients. Certain subtypes produce specific tumor markers: yolk sac tumors secrete alpha-fetoprotein (AFP), while choriocarcinomas produce human chorionic gonadotropin (hCG). Elevated levels of these markers aid in diagnosis and monitoring of treatment response. The current treatment approach typically involves surgical resection, often with fertility-sparing surgery (unilateral salpingo-oophorectomy) given the young age of most patients, followed by platinum-based combination chemotherapy, most commonly the BEP regimen (bleomycin, etoposide, and cisplatin). With modern treatment protocols, the prognosis has improved significantly, with high cure rates even in advanced-stage disease. Regular monitoring of serum tumor markers and imaging is essential for detecting recurrence. Prognosis varies by histological subtype and stage at diagnosis, but overall survival rates are favorable with appropriate multimodal therapy.
Also known as:
Sporadic
Usually appears on its own, not inherited from a parent
Variable
Can begin at different ages, from infancy through adulthood
Treatments
No FDA-approved treatments are currently listed for Malignant non-dysgerminomatous germ cell tumor of ovary.
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Specialists
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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
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Common questions about Malignant non-dysgerminomatous germ cell tumor of ovary
What is Malignant non-dysgerminomatous germ cell tumor of ovary?
Malignant non-dysgerminomatous germ cell tumors of the ovary are a group of rare, aggressive ovarian cancers that arise from primordial germ cells. This category encompasses several histological subtypes including yolk sac tumor (endodermal sinus tumor), immature teratoma, embryonal carcinoma, choriocarcinoma, and mixed germ cell tumors. These tumors are distinct from dysgerminomas and tend to occur predominantly in children, adolescents, and young women, typically during the reproductive years. These tumors primarily affect the ovary but can spread to other structures within the pelvis and a
How is Malignant non-dysgerminomatous germ cell tumor of ovary inherited?
Malignant non-dysgerminomatous germ cell tumor of ovary follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.