{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,7]],"date-time":"2026-07-07T00:33:47Z","timestamp":1783384427800,"version":"3.54.6"},"reference-count":37,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2025,5,20]],"date-time":"2025-05-20T00:00:00Z","timestamp":1747699200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2025,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Renal medullary carcinoma (RMC) is an aggressive tumor associated with sickle cell trait. Despite treatment advances for other rare renal tumors, RMC survival remains poor. We aimed to describe the contemporary management and survival of children and adults with RMC.<\/jats:p><\/jats:sec><jats:sec><jats:title>Procedure<\/jats:title><jats:p>In this multicenter retrospective cohort study, Pediatric Surgical Oncology Research Collaborative sites searched their databases for patients diagnosed with RMC (2000\u20132022). Descriptive statistics were calculated and survival analyses performed using Kaplan\u2013Meier and Cox regression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty\u2010four patients with RMC were identified. Median age was 19\u00a0years (IQR: 15\u201328; range: 7\u201352). Most were male (24\/34; 71%), Black (27\/32; 84%), had sickle cell trait or disease (30\/33; 91%), presented with metastatic disease (27\/34; 79%), and were symptomatic at presentation (32\/34; 94%). Median overall survival (OS) was 24\u00a0months from diagnosis (16\u00a0months for children, 28\u00a0months for adults, <jats:italic>p<\/jats:italic> = 0.6). Receipt of platinum\u2010based chemotherapy (23\/34; 68%) was associated with significantly higher OS than other regimens (35 vs. 5\u00a0months, <jats:italic>p<\/jats:italic>\u00a0&lt;\u00a00.001). Nephrectomy (24\/34; 71%) was associated with significantly improved OS compared with non\u2010operative management (34 vs. 7\u00a0months, <jats:italic>p<\/jats:italic> = 0.001). Immunotherapy, targeted therapy, or radiation therapy were not associated with significant differences in OS, nor were age, sex, race, sickle cell status, SMARCB1\/INI\u20101, stage, nephrectomy approach, retroperitoneal lymph node dissection, gross residual disease, margins, or tumor size.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>RMC survival remains poor despite newer therapies. Nephrectomy and platinum\u2010based chemotherapy should be considered in locally advanced and metastatic disease. Coordinated international cooperative group studies are needed to meaningfully improve RMC survival.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.31774","type":"journal-article","created":{"date-parts":[[2025,5,21]],"date-time":"2025-05-21T00:18:17Z","timestamp":1747786697000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Contemporary Biology, Management, and Outcomes of Renal Medullary Carcinoma in Children and Adults: A Pediatric Surgical Oncology Research Collaborative Study"],"prefix":"10.1002","volume":"72","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3685-2104","authenticated-orcid":false,"given":"Catherine B.","family":"Beckhorn","sequence":"first","affiliation":[{"name":"Duke University School of Medicine  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8803-8553","authenticated-orcid":false,"given":"Marcus M.","family":"Malek","sequence":"additional","affiliation":[{"name":"Division of Pediatric General and Thoracic Surgery UPMC Children's Hospital of Pittsburgh  Pittsburgh Pennsylvania USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harold N.","family":"Lovvorn","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery Vanderbilt University Medical Center  Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Harold J.","family":"Leraas","sequence":"additional","affiliation":[{"name":"Department of Surgery Duke University Medical Center  Durham North Carolina USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Katlyn G.","family":"McKay","sequence":"additional","affiliation":[{"name":"Department of Surgery Vanderbilt University Medical Center  Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nelly\u2010Ange T.","family":"Kontchou","sequence":"additional","affiliation":[{"name":"Department of Surgery Vanderbilt University Medical Center  Nashville Tennessee USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2561-7539","authenticated-orcid":false,"given":"Zachary J.","family":"Kastenberg","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Department of Surgery University of Utah Primary Children's Hospital  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7500-2631","authenticated-orcid":false,"given":"David W.","family":"Hoyt","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Department of Surgery University of Utah Primary Children's Hospital  Salt Lake City Utah USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jonathan P.","family":"Roach","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Department of Surgery University of Colorado School of Medicine Surgical Oncology Program Children's Hospital Colorado  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Emily K.","family":"Myers","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Department of Surgery University of Colorado School of Medicine Surgical Oncology Program Children's Hospital Colorado  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5785-3704","authenticated-orcid":false,"given":"Nicholas G.","family":"Cost","sequence":"additional","affiliation":[{"name":"Division of Urology Department of Surgery University of Colorado School of Medicine Surgical Oncology Program Children's Hospital Colorado  Aurora Colorado USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bhargava","family":"Mullapudi","sequence":"additional","affiliation":[{"name":"Children's Mercy Research Institute  Kansas City Kansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0004-1042-0073","authenticated-orcid":false,"given":"Charles R.","family":"Marchese","sequence":"additional","affiliation":[{"name":"Children's Mercy Research Institute  Kansas City Kansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amanda R.","family":"Jensen","sequence":"additional","affiliation":[{"name":"Children's Mercy Research Institute  Kansas City Kansas USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3323-6509","authenticated-orcid":false,"given":"Timothy B.","family":"Lautz","sequence":"additional","affiliation":[{"name":"Division of Pediatric Surgery Department of Surgery Ann and Robert H. 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