Benign metanephric tumor

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Overview

Benign metanephric tumor is a rare group of benign renal neoplasms that arise from embryonic kidney (metanephric) tissue. This category encompasses three histological subtypes: metanephric adenoma, metanephric adenofibroma, and metanephric stromal tumor. These tumors originate in the kidney and are composed of cells that resemble the developing fetal kidney (metanephros). Metanephric adenoma is the most common subtype and is typically discovered incidentally during imaging studies performed for other reasons, though some patients may present with an abdominal mass, flank pain, hematuria (blood in the urine), or polycythemia (elevated red blood cell count). Metanephric stromal tumors occur predominantly in young children and may be associated with abnormalities of the renal vasculature. These tumors are generally considered benign with an excellent prognosis. The primary challenge lies in distinguishing them from malignant renal tumors, particularly Wilms tumor in children or papillary renal cell carcinoma in adults, as they can share overlapping imaging and histological features. BRAF V600E mutations have been identified in a significant proportion of metanephric adenomas, which can aid in differential diagnosis. Treatment typically involves surgical excision, either partial or total nephrectomy, which is usually curative. Conservative management with active surveillance may be considered in select cases when the diagnosis is confident. Recurrence after complete surgical removal is exceedingly rare, and metastatic behavior has only been reported in exceptional cases.

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

1 available

XGEVA

denosumab· Amgen, Inc.

Prevention of skeletal-related events in patients with bone metastases from solid tumors

No actively recruiting trials found for Benign metanephric tumor at this time.

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Search ClinicalTrials.gov ↗Join the Benign metanephric tumor community →

No specialists are currently listed for Benign metanephric tumor.

View NORD Rare Disease Centers ↗Undiagnosed Disease Network ↗

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

Financial Resources

1 resources
XGEVA(denosumab)Amgen, Inc.

Travel Grants

No travel grants are currently matched to Benign metanephric tumor.

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Community

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Caregiver 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 Benign metanephric tumor

What is Benign metanephric tumor?

Benign metanephric tumor is a rare group of benign renal neoplasms that arise from embryonic kidney (metanephric) tissue. This category encompasses three histological subtypes: metanephric adenoma, metanephric adenofibroma, and metanephric stromal tumor. These tumors originate in the kidney and are composed of cells that resemble the developing fetal kidney (metanephros). Metanephric adenoma is the most common subtype and is typically discovered incidentally during imaging studies performed for other reasons, though some patients may present with an abdominal mass, flank pain, hematuria (blood

How is Benign metanephric tumor inherited?

Benign metanephric tumor follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

What treatment and support options exist for Benign metanephric tumor?

1 patient support program are currently tracked on UniteRare for Benign metanephric tumor. See the treatments and support programs sections for copay assistance, eligibility, and contact details.