{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,3]],"date-time":"2025-10-03T00:00:57Z","timestamp":1759449657285,"version":"build-2065373602"},"reference-count":46,"publisher":"Georg Thieme Verlag KG","issue":"01","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Klin Monbl Augenheilkd"],"published-print":{"date-parts":[[2024,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>\n               Background Solitary fibrous tumours are rare. The aim of this study is to describe the clinical features, therapy and outcome of affected patients and to identify factors associated with recurrence.<\/jats:p><jats:p>\n               Methods Retrospective study of a cohort of 20 patients who underwent surgery for orbital solitary fibrous tumour at the University Department of Oral and Maxillofacial Surgery between 2002 and 2023. Demographic, clinical, and therapeutic data as well as tumour follow-up results were collected. Tumour volume and molecular genetic mutations were retrospectively determined.<\/jats:p><jats:p>\n               Results The median patient age was 49.5 years at initial surgery. The left orbit was affected in 65% of cases. The most common clinical symptom was proptosis (80%). This was reported with a mean lateral difference of 3.9\u2009mm (range: 1\u200a\u2013\u200a10\u2009mm). The tumours were localised predominantly in the intra- and extraconal space, craniolateral quadrant and middle third. The median tumour volume was 7.66\u2009cm\u00b3 (range 2.15\u200a\u2013\u200a12.57\u2009cm\u00b3). In all patients, the diagnosis was made by pathological examination. All tumours investigated showed a NAB2-STAT6 mutation. The most frequently detected mutation was the fusion NAB2 exon 4 \u2013 STAT6 exon 2. All patients were initially managed with frontolateral orbitotomy. Incomplete resection (R1-status) occurred in 35% (n\u2009=\u20097). The recurrence rate was 25% (n\u2009=\u20095), with a median disease-free interval of 45.5 months (range 23\u200a\u2013\u200a130). 80% (n\u2009=\u20094) of recurrences were initially R1-resected.<\/jats:p><jats:p>\n               Conclusion Orbital solitary fibrous tumours are rare tumours and are clinically manifested by signs of displacement of orbital structures. Diagnosis is made by histology and immunohistochemistry and can be proven with the molecular genetic detection of the NAB2-STAT6 mutation. The therapy of choice is complete surgical resection. R1-resection is more likely in the intraconal location as well as in location in the posterior third of the orbit \u2013 due to difficult surgical accessibility. The greatest risk factor for the development of recurrence is incomplete surgical excision. Late recurrences are possible, which is why a long-term connection to a specialised clinic is necessary.<\/jats:p>","DOI":"10.1055\/a-2165-6550","type":"journal-article","created":{"date-parts":[[2023,11,15]],"date-time":"2023-11-15T19:19:15Z","timestamp":1700075955000},"page":"15-29","source":"Crossref","is-referenced-by-count":0,"title":["Solitary Fibrous Tumours of the Orbit \u2013 Clinical Pathology, Therapy and Prognosis"],"prefix":"10.1055","volume":"241","author":[{"given":"Chiara","family":"Meier","sequence":"additional","affiliation":[{"name":"Mund-, Kiefer- und Gesichtschirurgie, Universit\u00e4tsklinik f\u00fcr Mund-, Kiefer- & Gesichtschirurgie, Klinken Essen-Mitte, Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Anja","family":"Eckstein","sequence":"additional","affiliation":[{"name":"Orthoptik, Universit\u00e4ts-Augenklinik Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideo Andreas","family":"Baba","sequence":"additional","affiliation":[{"name":"Pathologie, Universit\u00e4tsklinikum Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lara Helena","family":"Sichward","sequence":"additional","affiliation":[{"name":"Pathologie, Universit\u00e4tsklinikum Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Florian","family":"Grabellus","sequence":"additional","affiliation":[{"name":"Pathologie, Zentrum f\u00fcr Pathologie Essen-Mitte, Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yan","family":"Li","sequence":"additional","affiliation":[{"name":"Diagnostische und Interventionelle Radiologie und Neuroradiologie, Universit\u00e4tsklinikum Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christoph David","family":"Ziegenfu\u00df","sequence":"additional","affiliation":[{"name":"Diagnostische und Interventionelle Radiologie und Neuroradiologie, Universit\u00e4tsklinikum Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christopher","family":"Mohr","sequence":"additional","affiliation":[{"name":"Mund-, Kiefer- und Gesichtschirurgie, Universit\u00e4tsklinik f\u00fcr Mund-, Kiefer- & Gesichtschirurgie, Klinken Essen-Mitte, Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Roman","family":"Pf\u00f6rtner","sequence":"additional","affiliation":[{"name":"Mund-, Kiefer- und Gesichtschirurgie, Universit\u00e4tsklinik f\u00fcr Mund-, Kiefer- & Gesichtschirurgie, Klinken Essen-Mitte, Essen, Deutschland"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"194","published-online":{"date-parts":[[2023,11,15]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/ajim.4700220103","article-title":"Primary neoplasms of the pleura. 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