LAUSR

OBJECTIVE To conduct a systematic review of the literature for patients with extra-pelvic deep endometriosis. DATA SOURCE A thorough search was performed through PubMed/MEDLINE, EMBASE and Cochrane databases. METHODS OF STUDY SELECTION Following PRISMA guidelines, in the last 20 years that reported on primary extra-pelvic endometriosis were included (PROSPERO registration number CRD42019125370). TABULATION AND INTEGRATION Initial search identified 5.465 studies and 179 studies, mostly case reports and series, were included. A total of 230 parietal (PE), 43 visceral (VE), 628 thoracic (TE), 6 central nerve system (CNS), 12 extra-pelvic muscle or nerves, and one nasal endometriosis papers were identified. RESULTS Abdominal endometriosis was divided into PE and VE.  PE lesions involved primary lesions of the abdominal wall, groin and perineum where presenting symptoms were palpable mass (99%), cyclic pain (71%) and cyclic bleeding (48%).  Pre-operative clinical suspicion was low and use of tissue diagnosis was indeterminate (25%), and a few (8%) malignancy suspected. Surgical treatment for PE included wide local excision (97%) with 5% recurrence and no complications.  Patients with VE involving the abdominal organs - kidneys, liver, pancreas, biliary tract - were treated surgically (86%) with both conservative (51%) and radical resection (49%), with 15% recurrence and 2 major complications reported. In patients with TE involving the diaphragm, pleura and lung, isolated and concomitant lesions occurred and favored the right side (80%).  Patients with TE presented with triad of catamenial pain, pneumothorax and hemoptysis. Thoracoscopic with resection followed by pleurodesis were the most common procedures performed with 29% recurrence.  Adjuvant medical therapy with GnRH was used in 15% of cases. Preoperative imaging with MRI was used in all cases of non-thoracic and non-abdominal endometriosis. Common symptoms were paresthesia and cyclic pain with radiation. Surgical resection was reported in 84% with improvement of symptoms. CONCLUSIONS Extra-pelvic endometriosis, traditionally thought to be rare, have been reported in a considerable number of cases. Heightened awareness and clinical suspicion of the disease, and multi-disciplinary approach is recommended to achieve prompt diagnosis and optimize patient outcomes. At this time, there are no comparative studies to provide recommendations regarding optimal diagnostic methods, treatment options and outcomes for endometriosis involving extra-pelvic sites.