{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,23]],"date-time":"2025-10-23T16:52:54Z","timestamp":1761238374925,"version":"3.41.2"},"reference-count":22,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2017,2,24]],"date-time":"2017-02-24T00:00:00Z","timestamp":1487894400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100007363","name":"Fondazione Citt\u00e0 della Speranza","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100007363","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTS) may rarely occur in visceral tissues, and little is known about their clinical history. The present study retrospectively analyzed a group of patients prospectively registered in Italian pediatric protocols conducted between 1979 and 2004.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Inclusion criteria for the study were as follows: a pathological diagnosis of \u201cadult\u2010type NRSTS,\u201d arising at visceral sites (lung\u2010pleurae, liver, kidney, and mesentery\u2010bowel); age under 18 years; no previous treatment except for primary surgery; available clinical data; and written consent.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Thirty cases with visceral NRSTS were collected and analyzed. Sites of origin were as follows: mesentery\u2010bowel in 12 cases, lung\u2010pleurae in 11, liver in 5, and kidney in 2. According to the Intergroup Rhabdomyosarcoma Study (IRS) surgical grouping system, patients were classified as follows: nine IRS group I, three group II, 12 group III, and six group IV. Patients were treated with a multimodal approach including surgery, radiotherapy, and\/or chemotherapy, according to their characteristics. For the series as a whole, the 5\u2010year event\u2010free and overall survival rates were 33.3% and 40.0%, respectively. The IRS group (reflecting the feasibility of initial complete resection) emerged as the main prognostic factor. Survival rates also correlated with tumor size and local invasiveness, histological subtype, and tumor sites (the worst outcome was seen for tumors arising in the lung and pleurae).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>This study confirmed that visceral NRSTS are aggressive tumors carrying a worse prognosis than pediatric NRSTS arising in soft tissues of the extremities. Local treatment remains the main challenge for these tumors.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.26490","type":"journal-article","created":{"date-parts":[[2017,2,24]],"date-time":"2017-02-24T07:42:56Z","timestamp":1487922176000},"source":"Crossref","is-referenced-by-count":6,"title":["Pediatric nonrhabdomyosarcoma soft tissue sarcomas arising at visceral sites"],"prefix":"10.1002","volume":"64","author":[{"given":"Andrea","family":"Ferrari","sequence":"first","affiliation":[{"name":"Pediatric Oncology Unit Fondazione IRCCS Istituto Nazionale Tumori  Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"Magni","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Unit Fondazione IRCCS Istituto Nazionale Tumori  Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Luca","family":"Bergamaschi","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Unit Fondazione IRCCS Istituto Nazionale Tumori  Milano Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giovanni","family":"Cecchetto","sequence":"additional","affiliation":[{"name":"Pediatric Surgery Padova University  Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rita","family":"Alaggio","sequence":"additional","affiliation":[{"name":"Pathology Department Padova University  Padova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giuseppe Maria","family":"Milano","sequence":"additional","affiliation":[{"name":"Hematology\/Oncology Ospedale Pediatrico Bambino Ges\u00f9 IRCCS  Roma Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Patrizia","family":"Bertolini","sequence":"additional","affiliation":[{"name":"Pediatric Hematology Oncology Azienda Ospedaliera Universitaria di Parma  Parma Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eleonora","family":"Basso","sequence":"additional","affiliation":[{"name":"Pediatric Onco\u2010Hematology Stem Cell Transplantation and Cellular Therapy Division, Regina Margherita Children's Hospital  Turin Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carla","family":"Manzitti","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hematology\/Oncology Giannina Gaslini Children's Hospital  Genova Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martina","family":"Di Martino","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Service Department of Pediatrics, Second University  Napoli Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nauga","family":"Giurici","sequence":"additional","affiliation":[{"name":"Pediatric Oncology Universit\u00e0 di Trieste  Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Fraia","family":"Melchionda","sequence":"additional","affiliation":[{"name":"Department of Pediatrics University of Bologna, S. 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