Case report - Unusual presentation of Embryonal Rhabdomyosarcoma

Abstract : An eight year old girl presented to us with left superior orbital mass. On clinical and radiological investigations we made a differential diagnosis of veno lymphatic malformation or a hemangioma. The patient was treated with systemic steroids. However, she did not improve and the proptosis and swelling increased rapidly. The rapidity forced us to intervene surgically. The histopathological diagnosis came as embryonal rhabdomyosarcoma. This case highlights the different form a rhabdomyosarcoma can present with and alerts the treating ophthalmologist to have high degree of suspicion.Keyword :1.Embryonal Rhabdomyosarcoma , 2. pediatric tumour 3. rapidly progressing , 4.orbital mass , 5. veno lymphatic malformationCase An 8 year old girl from Kerala presented with complaints of rapidly progressive swelling in left eye for past 1 week following a fall. On presentation, she had a visual acuity of 6/6 in the right eye and 6/12 in the left eye. Clinical examination of the left orbit revealed a soft, non-tender, non-pulsatile, non-reducible mass in the superior periorbital region causing dystopia. (figure 1) Spontaneous lid opening was absent. There was no conjunctival congestion or chemosis and rest of the anterior as well as posterior segment examination was within normal limits. Both pupils were brisk. Ocular motility showed a limitation in elevation in left eye.