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We describe a case of a young patient who presented with left shoulder pain, continuous fever, and unexplained weight loss as atypical initial manifestations of bilateral PRL, confirmed with the standard set of investigations. Furthermore, this article reviews the literature and discusses various aspects of PRL, including pathophysiology, presentation patterns, imaging and pathological characteristics, management, and prognosis. This paper serves to provide an update and aims to enhance the understanding of PRL. Timely diagnosis and treatment are imperative to achieve improved outcomes. Clinicians should maintain a high index of suspicion in order to prevent morbidity and mortality associated with this serious disease.<\/jats:p>","DOI":"10.1136\/bcr-2020-235076","type":"journal-article","created":{"date-parts":[[2020,6,18]],"date-time":"2020-06-18T06:19:01Z","timestamp":1592461141000},"page":"e235076","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":20,"title":["Primary renal lymphoma: a comprehensive review of the pathophysiology, clinical presentation, imaging features, management and prognosis"],"prefix":"10.1136","volume":"13","author":[{"given":"Syed Rizwan A","family":"Bokhari","sequence":"first","affiliation":[{"name":"Bassett Medical Center, Cooperstown, New York, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7576-7319","authenticated-orcid":false,"given":"Faisal","family":"Inayat","sequence":"additional","affiliation":[{"name":"Allama Iqbal Medical College, Lahore, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria R","family":"Bokhari","sequence":"additional","affiliation":[{"name":"Allama Iqbal Medical College, Lahore, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abeera","family":"Mansoor","sequence":"additional","affiliation":[{"name":"Doctors Hospital and Medical Center, Lahore, Pakistan"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"239","published-online":{"date-parts":[[2020,6,17]]},"reference":[{"key":"2025082709510852000_13.6.e235076.1","first-page":"584","article-title":"Primary renal lymphoma","volume":"18","author":"Ladha","year":"2008","journal-title":"J Coll Physicians Surg Pak"},{"key":"2025082709510852000_13.6.e235076.2","first-page":"273","article-title":"Primary renal non-Hodgkin's lymphoma - a difficult differential diagnosis","volume":"25","author":"Gellrich","year":"2002","journal-title":"Onkologie"},{"key":"2025082709510852000_13.6.e235076.3","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1096-9098(199703)64:3<207::AID-JSO6>3.0.CO;2-E"},{"key":"2025082709510852000_13.6.e235076.4","doi-asserted-by":"crossref","first-page":"759","DOI":"10.1148\/radiology.144.4.7111721","article-title":"Renal lymphoma: radiologic-pathologic correlation of 21 cases","volume":"144","author":"Hartman","year":"1982","journal-title":"Radiology"},{"key":"2025082709510852000_13.6.e235076.5","doi-asserted-by":"crossref","first-page":"239","DOI":"10.1097\/01.ju.0000148570.46368.c0","article-title":"Primary renal lymphoma: a rare neoplasm that may present as a primary renal mass","volume":"173","author":"Ahmad","year":"2005","journal-title":"J Urol"},{"key":"2025082709510852000_13.6.e235076.6","doi-asserted-by":"crossref","first-page":"191","DOI":"10.4274\/Tjh.03164","article-title":"A pediatric renal lymphoma case presenting with central nervous system findings","volume":"30","author":"Baran","year":"2013","journal-title":"Turk J Haematol"},{"key":"2025082709510852000_13.6.e235076.7","doi-asserted-by":"crossref","first-page":"838","DOI":"10.1016\/S0022-5347(17)69314-5","article-title":"Lymphosarcoma of the kidney","volume":"60","author":"Gibson","year":"1948","journal-title":"J Urol"},{"key":"2025082709510852000_13.6.e235076.8","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1980.97"},{"key":"2025082709510852000_13.6.e235076.9","first-page":"1","article-title":"Primary renal lymphoma: an unusual finding following radical nephrectomy","volume":"5","author":"Rissman","year":"2017","journal-title":"Clin Nephrol Case Stud"},{"key":"2025082709510852000_13.6.e235076.10","doi-asserted-by":"crossref","unstructured":"Taneja A , Kumar V , Chandra AB . 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