Multidisciplinary Management of Aggressive Orbital Rhabdomyosarcoma in an Adult Patient: A Case Report and Literature...

Embryonic rhabdomyosarcoma (RMS) is a common tumor in children. In adults, it is an extremely rare tumor. There are three histological subtypes: embryonic, alveolar, and pleomorphic RMS. Pleomorphic RMS is most common in adults. Orbital site is a common primary site in children. In adults, it is an unusual presentation site. Management of RMS is based on a multimodal approach, including surgery, radiotherapy, and chemotherapy. In the pediatric population, a multidisciplinary approach has been validated in prospective trials in Europe and the USA. In adults, there is no consensus on the management of these tumors in the absence of dedicated clinical trials. Management of adult RMS is based on pediatric guidelines and data from retrospective series of adults. The prognosis of RMS differs according to age. Adult RMS has worse survival than in children. We report a case of a 37-year-old female patient presenting with an orbital embryonic RMS. Treatment involved enucleation, but rapid local progression was observed. Chemotherapy was initiated, but evaluation after three cycles demonstrated significant tumor progression. Surgical intervention combined with radiotherapy was performed, followed by adjuvant chemotherapy. This case highlights the aggressive biology of embryonal RMS in adults and the role of a multimodal treatment approach.