Ethmoid Maxillo Orbital Alveolar Rhabdomyosarcoma: Case Report and Review of the Literature

Abstract

Introduction: Rhabdomyosarcoma is a soft tissue sarcoma, of unknown etiology. It is divided into two entities: embryonic rhabdomyosarcoma and alveolar rhabdomyosarcoma. The latter is common in adults and very aggressive. It’s a lymphophilic tumour with 15 to 50% lymph node involvement. Immunohistochemistry is crucial for differentiating an alveolar form an embryonic one. However molecular biology must be performed before any treatment. Management is multidimensional, combining chemotherapy, radiation therapy and surgery. The objective of this presentation is to show the aggressive character of alveolar rhabdomyosarcoma as well as the evolution, therapeutic strategies with a review of the literature. Case presentation: 27-year-old patient consults for swelling in the right eye associated with exophthalmia, pain and redness of the eye with a decrease in visual acuity. Clinical examination noted a mass of the right eye with exophthalmia, and the radiologic examination of CT and magnetic resonance imaging revealed an ethmoido-maxillo-orbital process. Histology and the immunohistochemistry of the biopsy confirmed that it is an alveolar rhabdomyosarcoma with expression of desmin and myogenin. The extension assessment came back negative. Neoadjuvant chemotherapy with Vincristine, Actinomycin, Cyclophosphamide, Ifosfamide and Etoposide and 54 Gy radiotherapy was performed with good clinical response, visual acuity in both eyes and radiological stability, and the patient was monitored. With a follow-up of 4 months, she presents a local relapse confirmed by an anatomical pathology examination with chemotherapy like Adriamycin, Cisplatin, Ifosfamide/ Adriamycin, Ifosfamide and a waiver of Right eye. After two other locoregional relapses, she was admitted to the palliative care unit. Conclusion: Alveolar rhabdomyosarcoma is an aggressive tumour that requires multimodal management. Despite the combination of chemotherapy, radiation therapy and surgery, the prognosis remains ....