{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,3]],"date-time":"2026-11-03T23:01:57Z","timestamp":1793746917632,"version":"4.6.1"},"reference-count":28,"publisher":"Wiley","issue":"16","license":[{"start":{"date-parts":[[2018,6,15]],"date-time":"2018-06-15T00:00:00Z","timestamp":1529020800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006058","name":"St. Baldrick's Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006058","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["acsjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Cancer"],"published-print":{"date-parts":[[2018,8,15]]},"abstract":"<jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Renal cell carcinomas (RCCs) are rare in young patients. Knowledge of their pathologic and molecular spectrum remains limited, and no prospective studies have been performed to date in this population. This study analyzes patients diagnosed with RCC who were prospectively enrolled in the AREN03B2 Children's Oncology Group (COG). The objective was to classify these tumors with the aid of focused genetic testing and to characterize their features.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>All tumors registered as RCC by central review were retrospectively re\u2010reviewed and underwent additional ancillary studies. Tumors were classified according to the 2016 World Health Organization classification system when possible.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>\n                      In total, 212 tumors were identified, and these were classified as microphthalmia transcription factor (MiT) translocation RCC (MiT\u2010RCC) (41.5%), papillary RCC (16.5%), renal medullary carcinoma (12.3%), chromophobe RCC (6.6%), clear cell RCC (3.3%), fumarate hydratase\u2010deficient RCC (1.4%), and succinate dehydrogenase\u2010deficient RCC (0.5%). Other subtypes included tuberous sclerosis\u2010associated RCC (4.2%), anaplastic lymphoma kinase (\n                      <jats:italic>ALK<\/jats:italic>\n                      )\u2010rearranged RCC (3.8%), thyroid\u2010like RCC (1.4%), myoepithelial carcinoma (0.9%), and unclassified (7.5%). MiT\u2010RCCs were classified as either transcription factor E3 (\n                      <jats:italic>TFE3<\/jats:italic>\n                      ) (93.2%) or EB (\n                      <jats:italic>TFEB<\/jats:italic>\n                      ) (6.8%) translocations, and characterization of fusion partners was possible in most tumors.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSIONS<\/jats:title>\n                    <jats:p>\n                      The current study delineates the frequency of distinct RCC subtypes in a large prospective series of young patients and contributes knowledge to the diagnostic, clinical, and genetic features of MiT\u2010RCC, the most common subtype among this age group. The identification of rare subtypes expands the spectrum of RCC in young patients, supporting the need for a thorough diagnostic workup. These studies may aid in the introduction of specific therapies for different RCC subtypes in the future.\n                      <jats:bold>\n                        <jats:italic>Cancer<\/jats:italic>\n                        2018\n                      <\/jats:bold>\n                      . \u00a9\n                      <jats:italic>2018 American Cancer Society<\/jats:italic>\n                      .\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/cncr.31578","type":"journal-article","created":{"date-parts":[[2018,6,15]],"date-time":"2018-06-15T14:02:09Z","timestamp":1529071329000},"page":"3381-3389","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":100,"title":["The classification of pediatric and young adult renal cell carcinomas registered on the children's oncology group (COG) protocol AREN03B2 after focused genetic testing"],"prefix":"10.1002","volume":"124","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3767-7261","authenticated-orcid":false,"given":"Mariana M.","family":"Cajaiba","sequence":"first","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. Lurie Children's Hospital of Chicago,` Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lisa M.","family":"Dyer","sequence":"additional","affiliation":[{"name":"Division of Human Genetics Cincinnati Children's Hospital Medical Center Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5181-116X","authenticated-orcid":false,"given":"James I.","family":"Geller","sequence":"additional","affiliation":[{"name":"Division of Oncology Cincinnati Children's Hospital Medical Center Cincinnati Ohio"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lawrence J.","family":"Jennings","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. Lurie Children's Hospital of Chicago,` Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David","family":"George","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. Lurie Children's Hospital of Chicago,` Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dawn","family":"Kirschmann","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. Lurie Children's Hospital of Chicago,` Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen M.","family":"Rohan","sequence":"additional","affiliation":[{"name":"Department of Pathology Colorado Pathology Consultants Denver Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nicholas G.","family":"Cost","sequence":"additional","affiliation":[{"name":"Department of Surgery University of Colorado Denver Colorado"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Geetika","family":"Khanna","sequence":"additional","affiliation":[{"name":"Department of Radiology Washington University School of Medicine St Louis Missouri"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth A.","family":"Mullen","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology Children's Hospital Boston Massachusetts"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey S.","family":"Dome","sequence":"additional","affiliation":[{"name":"Division of Oncology Children's National Medical Center Washington District of Columbia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Conrad V.","family":"Fernandez","sequence":"additional","affiliation":[{"name":"IWK Health Center Dalhousie University Halifax Nova Scotia Canada"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth J.","family":"Perlman","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine Ann and Robert H. Lurie Children's Hospital of Chicago,` Chicago Illinois"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2018,6,15]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.20548"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2006.05.010"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.29368"},{"key":"e_1_2_8_5_1","volume-title":"WHO Classification of Tumours of the Urinary System and Male Genital Organs","author":"Moch H","year":"2016"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.pas.0000131558.32412.40"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.22346"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1309\/98YE9E442AR7LX2X"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1177\/1066896909354337"},{"key":"e_1_2_8_10_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.semdp.2015.02.003"},{"key":"e_1_2_8_11_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000545"},{"key":"e_1_2_8_12_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000631"},{"key":"e_1_2_8_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e318299f0fb"},{"key":"e_1_2_8_14_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e3182613d8f"},{"key":"e_1_2_8_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0090-4295(02)02154-4"},{"key":"e_1_2_8_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000163"},{"key":"e_1_2_8_17_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000292"},{"key":"e_1_2_8_18_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000237"},{"key":"e_1_2_8_19_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000248"},{"key":"e_1_2_8_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22346"},{"key":"e_1_2_8_21_1","doi-asserted-by":"publisher","DOI":"10.1002\/gcc.22382"},{"key":"e_1_2_8_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0b013e31818cb8f5"},{"key":"e_1_2_8_23_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.25592"},{"key":"e_1_2_8_24_1","doi-asserted-by":"publisher","DOI":"10.1111\/his.13167"},{"key":"e_1_2_8_25_1","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.2016.204"},{"key":"e_1_2_8_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000837"},{"key":"e_1_2_8_27_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000835"},{"key":"e_1_2_8_28_1","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-200306000-00005"},{"key":"e_1_2_8_29_1","doi-asserted-by":"publisher","DOI":"10.1097\/PAS.0000000000000562"}],"container-title":["Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fcncr.31578","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.31578","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1002\/cncr.31578","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cncr.31578","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T22:20:31Z","timestamp":1761862831000},"score":1,"resource":{"primary":{"URL":"https:\/\/acsjournals.onlinelibrary.wiley.com\/doi\/10.1002\/cncr.31578"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,6,15]]},"references-count":28,"journal-issue":{"issue":"16","published-print":{"date-parts":[[2018,8,15]]}},"alternative-id":["10.1002\/cncr.31578"],"URL":"https:\/\/doi.org\/10.1002\/cncr.31578","archive":["Portico"],"relation":{},"ISSN":["0008-543X","1097-0142"],"issn-type":[{"value":"0008-543X","type":"print"},{"value":"1097-0142","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,6,15]]},"assertion":[{"value":"2018-02-08","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-05-07","order":2,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2018-06-15","order":3,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}