PMC3173912_01
Patient with left-sided proptosis
JPN-6-36-g001.jpg
PMC3173912_01_JPN-6-36-g001.jpg
F1
A 13-year-old girl presented with features of painless, progressively increasing proptosis of 3 months duration [Figure 1].
Axial CT image showing (a) extraconal mass lesion displacing the globe and (b) intracranial, extradural extension of the lesion compressing bilateral frontal lobes. (c) Coronal and (d) axial contrast MRI showing the lesion occupying the globe and bilateral frontal lobes
JPN-6-36-g002.jpg
PMC3173912_01_JPN-6-36-g002.jpg
F2
It had also infiltrated extradurally along the anterior skull base and was causing bilateral frontal lobe compression [Figure 2a and b]. T2-isointense lesion with homogenous contrast enhancement in the left orbit and with bifrontal, extradural extension [Figure 2c and d]. The rapid progression of proptosis necessitated an early surgery. The girl, therefore, underwent a bifrontal craniotomy and a brownish-black tumor was encountered compressing the dura covering both the frontal lobes from the extradural aspect.
Intaoperative images of (a) the extradural frontal lesion and (b) the intraorbital part of the lesion after removing the frontal part of the lesion
JPN-6-36-g003.jpg
PMC3173912_01_JPN-6-36-g003.jpg
F3
It was removed and the intraorbital, extraconal part of the tumor also excised [Figure 3a and b].
Microphotograph showing (a) Tumor cells disposed in sheets having round to oval cells, mildly anisomorphic nuclei, dispersed chromatin, inconspicuous nucleoli and scant amount of cytoplasm (hematoxylin and eosin stain); (b) Tumor cells are positive for CD 99 (immunohistochemical stain) and (c) Neuron-specific enolase (immunohistochemical stain)
JPN-6-36-g004.jpg
PMC3173912_01_JPN-6-36-g004.jpg
F4
The lesion was negative for synaptophysin and leukocyte common antigen (LCA) [Figure 4a-c].
The follow-up photograph after 1.5 months showing significant resolution of the proptosis
JPN-6-36-g005.jpg
PMC3173912_01_JPN-6-36-g005.jpg
F5
At a follow-up of 3 months, the proptosis had substantially subsided and the extraocular movements of the left eye had also recovered [Figure 5].