Orbital Rhabdomyosarcoma: Clinicodemographic Features and Outcomes from Turkey

Purpose: To evaluate the clinical features, radiologic, and histopathologic characteristics of patients with orbital rhabdomyosarcoma (RMS). Methods: The records of 14 patients with histologically proven orbital rhabdomyosarcoma between November 2012 and January 2024 were evaluated retrospectively. The age, gender, referring diagnosis, year of diagnosis, clinical features, histopathological results, radiology reports, follow-up period, management plans, and prognosis were obtained from patient electronic files and descriptively presented. Results: A total of 14 patients with a mean age of 12.64 ± 6.68 years were evaluated. Ten were males 71.43%, and four were females 28.57%. Six (42.85%) patients presented initially with proptosis as the main presenting complaint, and all of the patients had some degree of proptosis. Eleven patients (78.57%) had embryonal RMS, and three patients (21.42%) had alveolar RMS subtypes. The follow-up period ranged between 6 months and 6 years (26.36 ± 21.55 months). In 10 (71.42%) patients, there was a good response to treatment with no orbital recurrence or progression of the lesion during the follow-up period. Two patients had (14.28%) mortality during follow-up. Conclusion: Rhabdomyosarcoma management and diagnosis are important for preventing its spread and threatening patients’ life and visual survival. Orbital RMS is a rare but important diagnosis in the orbit, and differential diagnoses need to be considered because RMS may have presenting features resembling other inflammatory orbital conditions.