{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,28]],"date-time":"2026-04-28T16:04:09Z","timestamp":1777392249899,"version":"3.51.4"},"reference-count":27,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2022,4,23]],"date-time":"2022-04-23T00:00:00Z","timestamp":1650672000000},"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":[[2022,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background\/objectives<\/jats:title><jats:p>Rhabdomyosarcoma of the perianal\/perineal region (PRMS) is rare, with poor survival and limited understanding of the functional consequences of treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Design\/methods<\/jats:title><jats:p>International Society of Pediatric Oncology (SIOP) malignant mesenchymal tumor (MMT) 95, Italian RMS 96, and European paediatric Soft tissue sarcoma Study Group (EpSSG) RMS 2005 studies were interrogated to identify factors that impact survival; in RMS 2005, functional outcomes were analyzed.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Fifty patients (nonmetastatic) were identified, median age 6.4\u00a0years (range: 0.1\u201319.6): 29 male, 21 female. Tumors were &gt;5\u00a0cm in 33 patients. Histopathological subtype was alveolar in 35. Lymph nodes were involved in 23 patients. In RMS 2005, 16\/21 (76%) tested alveolar tumors had positive FOXO1 fusion status. Diagnostic biopsy was performed in 37. Primary resection (13) was complete (R0) in one. Delayed primary excision (16) was complete in three. Radiotherapy (RT) in 34\/50 patients included external beam (28), brachytherapy (3), and both (3). Nodal RT was given in 16\/23 N1 patients (70%). Median follow\u2010up of alive patients (29) was 84.1\u00a0months (range: 3.6\u2013221.1). Relapse or progression occurred in 24 patients (48%), 87% were fatal and most events (63%) were locoregional. Five\u2010year event\u2010free survival (EFS) was 47.8 (95% CI: 32.8\u201361.3), and 5\u2010year overall survival (OS) was 52.6 (95% CI: 36.7\u201366.2), with age \u226510\u00a0years and tumor size &gt;5\u00a0cm impacting 5\u2010year EFS and OS (<jats:italic>p<\/jats:italic>\u00a0&lt;\u00a0.05). Functional outcome data showed bowel, genito\u2010urinary, and psychological issues; fecal incontinence in four of 21 survivors, and urinary symptoms in two of 21.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>About 60% of patients with nonmetastatic PRMS survive; older patients and those with large tumors have the worst outcomes. Biopsy should be the initial procedure, and definitive local therapy individualized. Quality\u2010of\u2010life and functional studies are needed to better understand the consequences of treatment.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.29739","type":"journal-article","created":{"date-parts":[[2022,4,23]],"date-time":"2022-04-23T10:43:16Z","timestamp":1650710596000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":12,"title":["Perianal\/perineal rhabdomyosarcoma: Results of the SIOP MMT 95, Italian RMS 96, and EpSSG RMS 2005 studies"],"prefix":"10.1002","volume":"69","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-4499-0742","authenticated-orcid":false,"given":"Timothy","family":"Rogers","sequence":"first","affiliation":[{"name":"Department of Pediatric Surgery University Hospitals Bristol and Weston NHS Foundation Trust  Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ilaria","family":"Zanetti","sequence":"additional","affiliation":[{"name":"Hematology Oncology Division Department of Women's and Children's Health Padova University Hospital  Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatrice","family":"Coppadoro","sequence":"additional","affiliation":[{"name":"Hematology Oncology Division Department of Women's and Children's Health University of Padova  Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3385-2301","authenticated-orcid":false,"given":"H\u00e9l\u00e8ne","family":"Martelli","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University Paris\u2010Saclay Bic\u00eatre Hospital  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Meriel","family":"Jenney","sequence":"additional","affiliation":[{"name":"Department of Pediatric Oncology University Hospital of Wales  Cardiff UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0296-5207","authenticated-orcid":false,"given":"Veronique","family":"Minard\u2010Colin","sequence":"additional","affiliation":[{"name":"Department of Pediatric and Adolescent Oncology Gustave Roussy  Villejuif France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sheila E. J.","family":"Terwisscha van Scheltinga","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Clare","family":"Skerritt","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University Hospitals Bristol and Weston NHS Foundation Trust  Bristol UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4729-825X","authenticated-orcid":false,"given":"Raquel D\u00e1vila","family":"Fajardo","sequence":"additional","affiliation":[{"name":"Department of Radiation Oncology University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3487-3745","authenticated-orcid":false,"given":"Florent","family":"Gu\u00e9rin","sequence":"additional","affiliation":[{"name":"Department of Pediatric Surgery University Paris\u2010Saclay Bic\u00eatre Hospital  Paris France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anna","family":"Kelsey","sequence":"additional","affiliation":[{"name":"Department of Pathology Central Manchester University Hospitals  Manchester UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes H. M.","family":"Merks","sequence":"additional","affiliation":[{"name":"Princess M\u00e1xima Center for Pediatric Oncology  Utrecht The Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Henry","family":"Mandeville","sequence":"additional","affiliation":[{"name":"The Royal Marsden Hospital and the Institute of Cancer Research  Sutton UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Gabriela","family":"Guill\u00e9n","sequence":"additional","affiliation":[{"name":"Department of Paediatric Surgery Vall d'Hebron University Hospital  Barcelona Spain"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Heidi","family":"Glosli","sequence":"additional","affiliation":[{"name":"Department of Paediatric Research Division of Paediatric and Adolescent Medicine Oslo University Hospital  Oslo Norway"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Federica","family":"De Corti","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Unit, Department of Women's and Children's Health Padova University Hospital  Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4462-5523","authenticated-orcid":false,"given":"Gianni","family":"Bisogno","sequence":"additional","affiliation":[{"name":"Hematology Oncology Division Department of Women's and Children's Health University of Padova  Padua Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,4,23]]},"reference":[{"key":"e_1_2_9_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM199907293410507"},{"key":"e_1_2_9_3_1","doi-asserted-by":"publisher","DOI":"10.1038\/nm0210\u2010171"},{"key":"e_1_2_9_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19900615)65:12<2787::AID-CNCR2820651230>3.0.CO;2-K"},{"key":"e_1_2_9_5_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2005.05.3801"},{"key":"e_1_2_9_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ijrobp.2014.01.051"},{"key":"e_1_2_9_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.10441"},{"key":"e_1_2_9_8_1","doi-asserted-by":"publisher","DOI":"10.1200\/jco.2001.19.12.3091"},{"key":"e_1_2_9_9_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.40.3287"},{"key":"e_1_2_9_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470\u20102045(19)30617\u20105"},{"key":"e_1_2_9_11_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2007.14.7207"},{"key":"e_1_2_9_12_1","doi-asserted-by":"publisher","DOI":"10.1053\/jpsu.2003.50106"},{"key":"e_1_2_9_13_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e3182a6f320"},{"key":"e_1_2_9_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.2950170308"},{"key":"e_1_2_9_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/1097\u20100142(19950915)76:6<1073::AID\u2010CNCR2820760624>3.0"},{"key":"e_1_2_9_16_1","doi-asserted-by":"publisher","DOI":"10.1200\/JCO.2011.40.3287"},{"key":"e_1_2_9_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470\u20102045(18)30337\u20101"},{"key":"e_1_2_9_18_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1359\u20106349(03)90028\u20100"},{"key":"e_1_2_9_19_1","doi-asserted-by":"publisher","DOI":"10.1080\/09553008014551861"},{"key":"e_1_2_9_20_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejca.2008.10.026"},{"key":"e_1_2_9_21_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00247\u2010003\u20101067\u20106"},{"key":"e_1_2_9_22_1","first-page":"0","article-title":"Common Terminology Criteria for Adverse Events (CTCAE) v5.0","volume":"2009","author":"National Institute of Cancer","year":"2010","journal-title":"NIH Publ"},{"key":"e_1_2_9_23_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.317.7172.1572"},{"key":"e_1_2_9_24_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383\u2010005\u20101592\u20105"},{"key":"e_1_2_9_25_1","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.28701"},{"key":"e_1_2_9_26_1","doi-asserted-by":"publisher","DOI":"10.1097\/SLA.0b013e31824f1c21"},{"key":"e_1_2_9_27_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1470\u20102045(12)70236\u2010X"},{"key":"e_1_2_9_28_1","doi-asserted-by":"publisher","DOI":"10.1111\/codi.15812"}],"container-title":["Pediatric Blood &amp; 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