{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T18:35:06Z","timestamp":1787164506931,"version":"build-2736575974"},"reference-count":18,"publisher":"Oxford University Press (OUP)","issue":"7","license":[{"start":{"date-parts":[[2019,3,26]],"date-time":"2019-03-26T00:00:00Z","timestamp":1553558400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2019,7,1]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>The aim of this study is to add to the current knowledge regarding extracranial malignant rhabdoid tumor (MRT), a rare and highly aggressive tumor that occurs most commonly in infants and young children.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Patients and Methods<\/jats:title>\n                    <jats:p>A retrospective medical record review was conducted on 53 patients with pathologically confirmed MRT in Beijing Children's Hospital between January 2007 and October 2017.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Fifty-three patients were diagnosed with MRT at a median age of 16\u2009months, including 32 cases of malignant rhabdoid tumor of the kidney (MRTK) and 21 cases of extrarenal extracranial rhabdoid tumor (EERT). Fourteen (14\/32, 43.75%) patients with MRTK and five (5\/21, 23.81%) patients with EERT had metastases at diagnosis, and quite a few number of cases occurred tumor rupture (26.42%). Among the 53 patients, 40 (75.47%) patients died, 10 (18.87%) patients survived, and 3 patients (5.66%) were lost to follow-up. Among the 40 dead patients, 38 patients died from rapid progression of the disease or tumor recurrence, and 2 patients died of severe postoperative complications. Most of the recurrent or relapsed cases (94.11%) occurred within 8\u2009months, with a median time of 76\u2009days after diagnosis. The overall survival rates of 3\u2009years and 5\u2009years for the entire cohort were 23.71% and 18.44%, respectively. After survival analysis, it was clear that a younger age at diagnosis and distant stage patients had relatively poor outcomes. The effect of treatment was the most difficult to analyze because patients were not treated uniformly. Statistically significant differences in survival were noted among patients treated with standard chemotherapy, total resection, and radiotherapy.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Extracranial MRT is still a highly aggressive tumor in children. Younger patients and those suffering from metastatic disease were most likely to have a poor outcome because of rapid progression or recurrence of the tumor.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1634\/theoncologist.2018-0416","type":"journal-article","created":{"date-parts":[[2019,3,26]],"date-time":"2019-03-26T14:52:42Z","timestamp":1553611962000},"page":"e551-e558","source":"Crossref","is-referenced-by-count":39,"title":["Clinical and Prognostic Characteristics of 53 Cases of Extracranial Malignant Rhabdoid Tumor in Children. A Single-Institute Experience from 2007 to 2017"],"prefix":"10.1093","volume":"24","author":[{"given":"Haiyan","family":"Cheng","sequence":"first","affiliation":[{"name":"Department\u2009of Surgical Oncology, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shen","family":"Yang","sequence":"additional","affiliation":[{"name":"Department\u2009of Surgical Oncology, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Siyu","family":"Cai","sequence":"additional","affiliation":[{"name":"Center for Clinical Epidemiology & Evidence-Based Medicine, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoli","family":"Ma","sequence":"additional","affiliation":[{"name":"Hematology Oncology Center, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Qin","sequence":"additional","affiliation":[{"name":"Department\u2009of Surgical Oncology, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Weiping","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Urology Surgery, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Libing","family":"Fu","sequence":"additional","affiliation":[{"name":"Department of Pathology, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qi","family":"Zeng","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mingjie","family":"Wen","sequence":"additional","affiliation":[{"name":"Department of Immunology, School of Basic Medical Sciences, Beijing, People's Republic of China"},{"name":"Advanced Innovation Center for Human Brain Protection, Capital Medical University, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoxia","family":"Peng","sequence":"additional","affiliation":[{"name":"Center for Clinical Epidemiology & Evidence-Based Medicine, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Huanmin","family":"Wang","sequence":"additional","affiliation":[{"name":"Department\u2009of Surgical Oncology, Beijing Children's Hospital, National Center for Children's Health, Beijing, People's Republic of China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2019,3,26]]},"reference":[{"key":"2021122507241076100_R1","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1002\/cncr.23020","article-title":"Extracranial malignant rhabdoid tumors in childhood: The Childrens Hospital Los Angeles experience","volume":"110","author":"Madigan","year":"2007","journal-title":"Cancer"},{"key":"2021122507241076100_R2","doi-asserted-by":"crossref","first-page":"203","DOI":"10.1038\/28212","article-title":"Truncating mutations of hSNF5\/INI1 in aggressive paediatric 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