Extrapelvic endometriosis: clinical manifestations, diagnosis, and management

Extrapelvic endometriosis can be associated with significant morbidity. Diagnosis is often delayed due to atypical and nonspecific symptoms, as well as the rarity of endometriosis implants in most extrapelvic sites. This review will describe the clinical manifestations, diagnosis, and management of endometriosis involving the diaphragm, thoracic cavity, abdominal wall, extrapelvic viscera, and nervous system. While gastrointestinal and urinary tract endometriosis are the most common sites of extragenital endometriosis, these sites may be considered pelvic and will not be addressed in this review.

Endometriosis implants have been reported in nearly every organ system, including the thoracic cavity, abdominal wall, hollow and solid abdominal viscera, and central and peripheral nervous system. Presentation and management vary by location. Thoracic endometriosis most commonly presents with pneumothorax, and surgical management should involve a multidisciplinary team. Abdominal wall endometriosis classically presents with cyclic abdominal pain, mass, and history of abdominal surgery, although may arise without prior surgical history; while surgical excision remains the mainstay of treatment, multiple local treatment modalities are emerging. Other sites of extrapelvic endometriosis are rare, and further research is needed on optimal management strategies.

Extrapelvic endometriosis should be considered in the differential diagnosis in patients with unusual catamenial symptoms. Multidisciplinary teams should be engaged to provide optimal care.