{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,9]],"date-time":"2026-04-09T22:31:48Z","timestamp":1775773908433,"version":"3.50.1"},"reference-count":20,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2024,12,12]],"date-time":"2024-12-12T00:00:00Z","timestamp":1733961600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Orbital rhabdomyosarcoma (RMS) is often limited to the orbital cavity and has a favorable prognosis. In some cases, the tumor can erode the orbital bone and behave as a parameningeal RMS (PM-RMS); thus, it is treated more intensively. However, the current protocols do not provide any guidance on how to consider different grades of bone erosion (BE) that can vary widely, hampering a uniform classification and the subsequent treatment assignment. With the aim of clarifying the role of BE as a risk factor, we analyzed patients with orbital RMS included in the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG) protocol.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analyzed the radiological reports of 199 patients with orbital RMS (PM or not) and defined three grades of BE: minimal (thinning of the bone), moderate (focal bone lysis), and extensive (complete cortical destruction).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>BE was present in 55 of the 199 (27.6%) patients, which was classified as minimal in 27, moderate in 7, and extensive in 21. Tumors with extensive BE were more frequently large (&amp;gt;5 cm, <jats:italic>p<\/jats:italic> = 0.0008) and invasive (T2, <jats:italic>p<\/jats:italic> = 0.001). With a median follow-up of 70.4 months (range = 7.1\u2013167.7), a total of 183 patients are alive, with 5-year event-free survival (EFS) and overall survival (OS) rates of 76% (95%CI = 69.2\u201381.3) and 92% (95%CI = 86.7\u201394.8), respectively. Patients without any BE had better OS (95% <jats:italic>vs<\/jats:italic>. 81%, <jats:italic>p<\/jats:italic> = 0.001), but not EFS. Patients with no\/minimal\/moderate BE had better EFS and OS compared with patients with extensive BE [EFS of 78.1 (95%CI = 71.1\u201383.5) <jats:italic>vs<\/jats:italic>. 57.1 (95%CI = 33.8\u201374.9), <jats:italic>p<\/jats:italic> = 0.0114, respectively, and OS of 94.0 (95%CI = 89.2\u201396.8) <jats:italic>vs<\/jats:italic>. 71.1 (95%CI = 46.6\u201385.9), <jats:italic>p<\/jats:italic> &amp;lt; 0.0001, respectively]. Events and metastatic relapses (in all cases CNS\/meningeal) were more frequent in patients with extensive BE.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Only those patients with orbital RMS and extensive BE should be considered as PM and should be treated accordingly.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fonc.2024.1497193","type":"journal-article","created":{"date-parts":[[2024,12,12]],"date-time":"2024-12-12T06:21:35Z","timestamp":1733984495000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Prognostic role of bone erosion in orbital RMS: a report from the European Pediatric Soft Tissue Sarcoma Study Group (EpSSG)"],"prefix":"10.3389","volume":"14","author":[{"given":"Daniela","family":"Di Carlo","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giulia","family":"Fichera","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Veronique","family":"Minard-Colin","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beatrice","family":"Coppadoro","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Daniel","family":"Orbach","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alison","family":"Cameron","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Monica","family":"Albiac Ramos","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Myriam","family":"Ben Arush","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes H. 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