Case report - Unusual presentation of Embryonal Rhabdomyosarcoma

Abstract

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 malformation-
Case 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.
MRI done elsewhere previously revealed a 2.4×1.2×2.0 cm
sized solid lesion in extra conal compartment of left orbit
superiorly with inferior displacement of superior rectus muscle
and left globe. The lesion was well defined with intensely
homogenous
enhancement. (figure 2&3) Haemangioma and lymphangioma
were considered in the differential diagnosis. CT scan with
contrast showed a poorly enhancing hypodense lesion involving
superior quadrant of left orbit and blends with superior orbital
tissues and the epicenter from upper eyelid originating from sub
periosteal zone causing downward displacement of globe. Size
of the lesion measured 3.6×2.6×2.4cms suggestive of a venolymphatic
malformation with bleeding.