Extrapelvic endometriosis

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ORPHA:137820N80.0N80.1N80.2
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2FDA treatments9Specialists8Treatment centers1Financial resources

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Overview

Extrapelvic endometriosis is a rare form of endometriosis in which endometrial-like tissue (tissue resembling the lining of the uterus) is found outside the pelvic cavity. While classic endometriosis typically involves the ovaries, fallopian tubes, and pelvic peritoneum, extrapelvic endometriosis can affect a wide range of organ systems including the gastrointestinal tract (bowel, appendix, rectum), urinary tract (bladder, ureters, kidneys), thoracic cavity (lungs, diaphragm — sometimes called thoracic endometriosis or catamenial pneumothorax), abdominal wall (particularly in surgical scars such as cesarean section scars), skin, and rarely the central nervous system or extremities. The condition predominantly affects women of reproductive age. Symptoms of extrapelvic endometriosis vary widely depending on the site of involvement and often follow a cyclical pattern corresponding to the menstrual cycle. Patients may experience chronic pain at the affected site, cyclical bleeding (such as hemoptysis in thoracic endometriosis or hematuria in urinary tract involvement), bowel obstruction, or palpable masses in the abdominal wall. Diagnosis can be challenging due to the diverse and often atypical presentations, frequently requiring imaging studies (MRI, CT, ultrasound) and histological confirmation through biopsy or surgical excision. Treatment approaches include hormonal therapies aimed at suppressing estrogen production and menstruation (such as GnRH agonists, progestins, and combined oral contraceptives), which can help control symptoms and reduce lesion activity. Surgical excision of extrapelvic endometriotic implants is often necessary, particularly for symptomatic masses, bowel or urinary tract obstruction, or thoracic disease causing recurrent pneumothorax. A multidisciplinary approach involving gynecologists, surgeons, pulmonologists, or urologists is often required depending on the organ system involved. There is currently no cure, and recurrence after treatment is possible.

Also known as:

Inheritance

Multifactorial

Caused by a mix of several genes and environmental factors

Age of Onset

Adult

Begins in adulthood (age 18 or older)

Orphanet ↗NORD ↗

Treatments

2 available

Synarel Nasal Solution

Nafarelin acetate· Syntex (USA), Inc.

SYNAREL is indicated for management of endometriosis, including pain relief and reduction of endometriotic lesions.

Danazol

DANAZOL· Chartwell RX, LLC.■ Boxed Warning

Danazol capsules are indicated for the treatment of endometriosis amenable to hormonal management

No actively recruiting trials found for Extrapelvic endometriosis at this time.

New trials open frequently. Follow this disease to get notified.

Search ClinicalTrials.gov ↗Join the Extrapelvic endometriosis community →

Specialists

9 foundView all specialists →
GS
Giovanni Scambia
Specialist
PI on 2 active trials
AH
Abdulkarim Hasan
Specialist
PI on 1 active trial
FC
Francesco Cosentino
Specialist
PI on 1 active trial
AT
Anna Thanasa
Specialist
3 Extrapelvic endometriosis publications
ET
Efthymia Thanasa
Specialist
3 Extrapelvic endometriosis publications
EK
Evangelos Kamaretsos
Specialist
3 Extrapelvic endometriosis publications
IT
Ioannis Thanasas
Specialist
3 Extrapelvic endometriosis publications
GK
Gerasimos Kontogeorgis
Specialist
2 Extrapelvic endometriosis publications
IP
Ioannis Paraoulakis
Specialist
2 Extrapelvic endometriosis publications

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
Danazol(DANAZOL)Chartwell RX, LLC.

Travel Grants

No travel grants are currently matched to Extrapelvic endometriosis.

Search all travel grants →NORD Financial Assistance ↗

Community

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Latest news about Extrapelvic endometriosis

No recent news articles for Extrapelvic endometriosis.

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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 Extrapelvic endometriosis

What is Extrapelvic endometriosis?

Extrapelvic endometriosis is a rare form of endometriosis in which endometrial-like tissue (tissue resembling the lining of the uterus) is found outside the pelvic cavity. While classic endometriosis typically involves the ovaries, fallopian tubes, and pelvic peritoneum, extrapelvic endometriosis can affect a wide range of organ systems including the gastrointestinal tract (bowel, appendix, rectum), urinary tract (bladder, ureters, kidneys), thoracic cavity (lungs, diaphragm — sometimes called thoracic endometriosis or catamenial pneumothorax), abdominal wall (particularly in surgical scars su

How is Extrapelvic endometriosis inherited?

Extrapelvic endometriosis follows a multifactorial inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.

At what age does Extrapelvic endometriosis typically begin?

Typical onset of Extrapelvic endometriosis is adult. Age of onset can vary across affected individuals.

Which specialists treat Extrapelvic endometriosis?

9 specialists and care centers treating Extrapelvic endometriosis are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.

What treatment and support options exist for Extrapelvic endometriosis?

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