Malignant non-dysgerminomatous germ cell tumor of ovary

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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.

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Inheritance

Sporadic

Usually appears on its own, not inherited from a parent

Age of Onset

Variable

Can begin at different ages, from infancy through adulthood

Orphanet ↗NORD ↗

Treatments

No FDA-approved treatments are currently listed for Malignant non-dysgerminomatous germ cell tumor of ovary.

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No actively recruiting trials found for Malignant non-dysgerminomatous germ cell tumor of ovary at this time.

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No specialists are currently listed for Malignant non-dysgerminomatous germ cell tumor of ovary.

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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

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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.