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Clinical presentation and therapy may differ based on location, size, and morphology. A comprehensive and updated systematic review of the literature is lacking.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A systematic review of the literature was performed according to PRISMA guidelines. Pubmed, Embase, Cochrane, and Medline databases were consulted using MESH keywords. Non\u2010English written articles and abstracts were excluded. Sex, age, symptoms at presentation, diagnosis, tumor location and size, surgical approach and technique of excision, pathology, and morphology were extracted and recorded in an electronic database.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Sixty\u2010seven studies for a total of 239 patients with esophageal lipoma or liposarcoma were included in the qualitative analysis. Among 176 patients with benign lipoma, the median age was 55. The main symptoms were dysphagia (64.2%), transoral polyp regurgitation (32.4%), and globus sensation (22.7%). The majority of lipomas (85.7%) were intraluminal polyps, with a stalk originating from the upper esophagus. Overall, 165 patients underwent excision of the mass through open surgery (65.5%), endoscopy (27.9%), or laparoscopy\/thoracoscopy (3.6%). Only 5 (3%) of patients required esophagectomy. Of the 11 untreated patients with an intraluminal polyp, 7 died from asphyxia. Overall, liposarcoma was diagnosed in 63 patients, and 12 (19%) underwent esophagectomy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Esophageal lipomatous tumors are rare but potentially lethal when are intraluminal and originate from the cervical esophagus. Modern radiological imaging has improved diagnostic accuracy. 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