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:
Multifactorial
Caused by a mix of several genes and environmental factors
Adult
Begins in adulthood (age 18 or older)
Treatments
2 availableSynarel Nasal Solution
SYNAREL is indicated for management of endometriosis, including pain relief and reduction of endometriotic lesions.
Danazol
Danazol capsules are indicated for the treatment of endometriosis amenable to hormonal management
Clinical Trials
View all trials with filters →No actively recruiting trials found for Extrapelvic endometriosis at this time.
New trials open frequently. Follow this disease to get notified.
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
Financial Resources
1 resourcesTravel Grants
No travel grants are currently matched to Extrapelvic endometriosis.
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 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.