Orbital embryonal rhabdomyosarcoma: a case-based update.

Abstract

Background: Rhabdomyosarcoma (RMS) is the most prevalent soft tissue sarcoma in children, with approximately 30% of head and neck RMS occurring in the orbit. The management of orbital RMS is complex, requiring a multidisciplinary approach and careful surgical planning. The objective of the present paper is to provide the neurosurgeon with an update on this challenging tumor.

Case description: A 12-year-old boy was admitted to our department after the onset of diplopia in his right eye, associated with quickly worsening ipsilateral eyelid edema and mild proptosis. MRI revealed an anterior superomedial, extraconal, and intraorbital expansive lesion. Complete tumor removal was performed through a trans-orbital approach. Histological diagnosis of embryonal RMS was done. The patient underwent radio-chemotherapy in accordance with the EpSSG RMS2005 subgroup C protocol. The patient is alive and disease-free.

Discussion and conclusion: The intricacies of treating orbital tumors necessitate meticulous planning to safeguard vital structures while guaranteeing comprehensive oncological treatment. A multidisciplinary approach with a specific protocol depending on the location and characteristics of the tumor is required. The available treatment options include surgical intervention or combination therapies, such as chemotherapy and radiotherapy. The successful management of these diseases depends on the careful coordination and application of the available techniques.