Abstract
Orbital Rhabdomyosarcoma: Current Perspectives
Highlights
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood, orbital RMS presented 8% of all soft tissue sarcoma of the head and neck
Most cases of orbital RMS were diagnosed before 16 years and the median age is between 5-7 years, some cases were described in elderly patients [4]
The standard of care for orbital RMS in the past was complete resection enucleation or exenteration of the tumor but due to the poor overall survival (OS) about 25-30% observed by sugery alone and in attempt to improve this outcome, the North American Intergroup Rhabdomyosarcoma Study Group (IRSG) and European cooperative groups add adjuvant chemotherapy and radiotherapy in cooperative group trials, the OS was improved to around 90% [14,15,16,17]
Summary
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in childhood, orbital RMS presented 8% of all soft tissue sarcoma of the head and neck. The embryonal subtype is the most common orbital RMS is associated with a good prognosis than others. Treatment of orbital RMS is multimodal, including surgical excision with or without radiotherapy and chemotherapy.
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