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At least a part of the retroperitoneal fibrosis, which was originally classified as idiopathic, seems to be assigned to IgG4-related disease. Lymphangiomas are benign, cystic tumors that rarely occur in adults. However, there is no firm association with IgG4-related disease described in the literature to date. This report is about a patient suffering from acute renal failure due to a giant retroperitoneal cyst. Surgical resection remains incomplete in the iliac vessel area due to severe fibrosis and histology revealed features of both lymphangioma and IgG4<jats:sup>+<\/jats:sup> fibrosis. The case description is followed by a brief overview of IgG4-related disease and a consideration of whether lymphangiomas might be assigned to this topic. <\/jats:p>","DOI":"10.1177\/2050313x211016993","type":"journal-article","created":{"date-parts":[[2021,5,20]],"date-time":"2021-05-20T07:19:39Z","timestamp":1621495179000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":1,"title":["A case of giant retroperitoneal lymphangioma and IgG4-positive fibrosis: Causality or coincidence?"],"prefix":"10.1177","volume":"9","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0273-509X","authenticated-orcid":false,"given":"Kim Rouven","family":"Liedtke","sequence":"first","affiliation":[{"name":"Department of General, Visceral, Thoracic and Vascular Surgery, Greifswald University Medical Centre, Greifswald, Germany"},{"name":"Department of Trauma and Orthopedic Surgery, Experimental Trauma Surgery, University Medical Center 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