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The majority of cases are of the embryonal subtype, which carries a favourable prognosis when promptly diagnosed and appropriately managed. Advances in molecular profiling have further refined risk stratification, distinguishing PAX3\/7::FOXO1 fusion\u2010negative embryonal from fusion\u2010positive alveolar RMS. Imaging, particularly MRI, plays a central role in diagnosis and tumour extent. Current biopsy recommendations favour open incisional procedures for orbital RMS. Treatment follows risk\u2010adapted protocols incorporating systemic chemotherapy with radiotherapy for local control. Emerging studies show the benefit of novel radiotherapeutic approaches that have high control rates but lower toxicity, including brachytherapy in the AMORE protocol. Despite overall excellent survival rates, challenges remain in reducing long\u2010term morbidity and personalising treatment through genetic and molecular knowledge. This review offers current recommendations and highlights the importance of a multi\u2010disciplinary approach in the diagnosis, staging and treatment of orbital RMS.<\/jats:p>","DOI":"10.1111\/ceo.70075","type":"journal-article","created":{"date-parts":[[2026,2,16]],"date-time":"2026-02-16T23:34:25Z","timestamp":1771284865000},"page":"398-409","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":1,"title":["Orbital Rhabdomyosarcoma: A Comprehensive Review of Clinical Features, Molecular Advances and Current Management"],"prefix":"10.1111","volume":"54","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8817-371X","authenticated-orcid":false,"given":"Kamil Gabri\u00ebl","family":"Laban","sequence":"first","affiliation":[{"name":"Department of Ophthalmology University Medical Center Utrecht  Utrecht the Netherlands"},{"name":"Orbital Center, Department of Ophthalmology Amsterdam University Medical Centers  Amsterdam the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nur","family":"Khatib","sequence":"additional","affiliation":[{"name":"Orbital Center, Department of Ophthalmology Amsterdam University Medical Centers  Amsterdam the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Johannes Hendrikus Maria","family":"Merks","sequence":"additional","affiliation":[{"name":"Princess Maxima Center for Pediatric Oncology  Utrecht the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Peerooz","family":"Saeed","sequence":"additional","affiliation":[{"name":"Orbital Center, Department of Ophthalmology Amsterdam University Medical Centers  Amsterdam the Netherlands"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2026,2,16]]},"reference":[{"key":"e_1_2_15_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ccr.2011.01.044"},{"key":"e_1_2_15_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00417-024-06685-6"},{"key":"e_1_2_15_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/cncr.35974"},{"key":"e_1_2_15_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/cam4.5211"},{"key":"e_1_2_15_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-0142(19970915)80:6<1165::AID-CNCR21>3.0.CO;2-5"},{"key":"e_1_2_15_7_1","first-page":"139","article-title":"Rhabdomyosarcoma of the Orbit","volume":"39","author":"Reese A. 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