Orbital Alveolar Rhabdomyosarcoma –Unusual Presentation and Dreadful Experience

Orbital Alveolar Rhabdomyosarcoma –Unusual Presentation and Dreadful

Abstract

Introduction: Orbital Rhabdomyosarcoma (RMS) is one of the few life-threatening diseases seen by ophthalmologists and prompt diagnosis and treatment can save the life of the affected patient. Survival after treatment of RMS has improved from 25% in 1970 to 70% in 1991.
Case Report: A 15 year girl presented with 5.5cmx5cmx4.8cm multi-lobulated mass lesion with necrosis in right medial orbital fossa and pre-auricular and submandibular lymphadenopathy. MRI suggested possibility of malignant soft tissue tumor with extensive spread into sinuses and necessitated tissue diagnosis. Incisional biopsy revealed alveolar rhabdmyosarcoma. Due to extensive involvement of sinuses patient was advised primary chemoradiation therapy. Despite full regimen of primary chemo-radiation, within 4 months it spread to involve whole of the face and other eye pursuing a devastating course.
Conclusion: This is a rare case of orbital alveolar Rhabdomyosarcoma in a 15 year female with poor response to chemo-radiation.