OBSOLETE: Borderline epithelial tumor of ovary

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ORPHA:206473
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Overview

Borderline epithelial tumor of ovary, also known as borderline ovarian tumor (BOT) or ovarian tumor of low malignant potential, is a classification that has been marked as OBSOLETE in the Orphanet database (Orphanet code 206473). These tumors represent a distinct category of ovarian neoplasms that exhibit atypical epithelial proliferation greater than that seen in benign tumors but without the destructive stromal invasion characteristic of frank ovarian carcinoma. They primarily affect the female reproductive system, specifically the ovaries, and can be of serous, mucinous, endometrioid, clear cell, or transitional (Brenner) histological subtypes, with serous and mucinous being the most common. Borderline ovarian tumors typically present in women of reproductive age, often at an earlier stage than invasive ovarian cancers. Symptoms may include pelvic or abdominal pain, abdominal distension, bloating, and occasionally an incidentally discovered adnexal mass on imaging. Some patients may be asymptomatic. The prognosis is generally favorable compared to invasive ovarian carcinoma, with high survival rates, particularly when diagnosed at early stages. Treatment primarily involves surgical management, with the extent of surgery depending on the patient's age, desire for fertility preservation, and the stage of disease. Conservative fertility-sparing surgery (such as unilateral salpingo-oophorectomy or cystectomy) may be considered in younger patients, while complete surgical staging including bilateral salpingo-oophorectomy and hysterectomy may be recommended for patients who have completed childbearing. The role of adjuvant chemotherapy remains controversial and is generally not recommended for most borderline tumors. Long-term follow-up is important due to the possibility of late recurrence. The obsolete status of this Orphanet entry likely reflects reclassification or merging into a broader or updated disease category within the database.

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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 ↗

Treatments

No FDA-approved treatments are currently listed for OBSOLETE: Borderline epithelial tumor of ovary.

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No specialists are currently listed for OBSOLETE: Borderline epithelial 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 OBSOLETE: Borderline epithelial tumor of ovary

What is OBSOLETE: Borderline epithelial tumor of ovary?

Borderline epithelial tumor of ovary, also known as borderline ovarian tumor (BOT) or ovarian tumor of low malignant potential, is a classification that has been marked as OBSOLETE in the Orphanet database (Orphanet code 206473). These tumors represent a distinct category of ovarian neoplasms that exhibit atypical epithelial proliferation greater than that seen in benign tumors but without the destructive stromal invasion characteristic of frank ovarian carcinoma. They primarily affect the female reproductive system, specifically the ovaries, and can be of serous, mucinous, endometrioid, clear

How is OBSOLETE: Borderline epithelial tumor of ovary inherited?

OBSOLETE: Borderline epithelial tumor of ovary follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does OBSOLETE: Borderline epithelial tumor of ovary typically begin?

Typical onset of OBSOLETE: Borderline epithelial tumor of ovary is adult. Age of onset can vary across affected individuals.