Successful Local Control of Orbital ASPS Using VMAT-Based Adjuvant Radiotherapy with Simultaneous Integrated Boost: A 3-Year Follow-Up Case Report
Year: 2025
Type: article
Study design: Case Report
Abstract: : Orbital Alveolar Soft Part Sarcoma (ASPS) is an extremely rare malignancy, with limited case reports. Diagnosis is challenging owing to its slow-growing nature and non-specific symptoms. Furthermore, standardized treatment protocols are lacking, and the long-term outcomes remain poorly understood. We aimed to address these gaps by presenting a rare case of orbital ASPS and outlining the diagnostic challenges, treatment approach, and long-term follow-up to inform the clinical management. A 28-year-old male presented with diplopia, restricted ocular movements, and orbital swelling. Magnetic resonance imaging revealed a 3.81 × 1.99-cm mass adjacent to the left medial rectus muscle, which was histopathologically confirmed as ASPS. A personalized therapeutic approach involving surgery and radiotherapy achieved stable disease, preserved vision, and no recurrence over a 3-year follow-up period. These findings contribute to the existing body of knowledge regarding the clinical management of orbital ASPS, underscoring the importance of individualized treatment strategies. Routine long-term surveillance and multidisciplinary care are essential in the management of rare cases. This case further emphasizes the need for continued research and documentation to enhance the diagnostic accuracy and refine the treatment guidelines for ASPS. Plain Language Summary: Alveolar Soft Part Sarcoma (ASPS) is a rare cancer that can appear in the soft tissues of the body. It is even more uncommon and difficult to diagnose when it occurs in the eye socket (orbit) because the tumor grows slowly, and the symptoms are vague, such as double vision and eye swelling. There are very few reports on this condition and there is no standard treatment plan. In this report, we describe the case of a 28-year-old man who developed orbital ASPS. The patient experienced double vision, limited eye movement, and swelling in one eye. A magnetic resonance imaging (MRI) scan showed a tumor near the eye muscle, which was confirmed to be ASPS through tissue testing. The patient was treated with a combination of surgery and targeted radiotherapy. Over a 3-year follow-up period, his vision remained stable and the cancer did not return. This case shows that careful planning using advanced radiotherapy techniques, along with long-term follow-up and regular eye examinations, can help to control this rare cancer while protecting vision. Our experience highlights the importance of a personalized approach involving a team of medical specialists. Improved radiotherapy methods, such as proton therapy, may further reduce side effects and improve outcomes. More research is needed to better understand this rare disease and develop clearer treatment guidelines. Keywords: long-term surveillance, orbital ASPS, personalized treatment, radiotherapy, recurrence management, TFE3 gene
Authors: Hind Albadrani, Lulwah Abduljabbar
Language: English
Subfield: Ophthalmology
Field: Medicine
Domain: Health Sciences
Open Access status: gold
Estimated APC paid: $2,320
OpenAlex ID: W4417187841