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We report herein a 66-year-old patient with symptomatic retroperitoneal fibrosis leading to bilateral hydronephrosis and renal failure, in whom, after a complex diagnostic work-up and protracted clinical course, a B-cell non-Hodgkin lymphoma in the retroperitoneal space and several vertebral bodies was identified. The patient was treated with radiation therapy and weekly rituximab infusions, with resolution of hydronephrosis and lower back pain. We include a thorough literature review on etiopathogenesis, diagnosis, therapy, and prognosis of retroperitoneal fibrosis. A meticulous search for malignancy is necessary in this rare condition that, if positive, may have significant therapeutic and prognostic implications.<\/jats:p>","DOI":"10.1177\/1078155214543279","type":"journal-article","created":{"date-parts":[[2014,7,10]],"date-time":"2014-07-10T20:25:39Z","timestamp":1405023939000},"page":"179-185","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":11,"title":["Retroperitoneal fibrosis due to B-cell non-Hodgkin lymphoma: Responding to rituximab!"],"prefix":"10.1177","volume":"22","author":[{"given":"Juliana","family":"Alvarez Argote","sequence":"first","affiliation":[{"name":"Department of Internal Medicine, University of Connecticut, Farmington, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank A","family":"Bauer","sequence":"additional","affiliation":[{"name":"Department of Pathology, St. Francis Hospital and Medical Center, Hartford, 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