PMC6935963_01
Computed tomography showed the left intraconal orbital tumor compressing the globe with resultant exophthalmos (a). T2-weighted magnetic resonance imaging (MRI) showed a hyperintense tumor (b). Gadolinium-enhanced fat suppression T1-weighted MRI showed a heterogeneous enhancement (c). VasoCT showed some feeders from the ophthalmic artery (d).
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F1
Computed tomography showed an apparent proptosis of the left eye [Figure 1a]. Magnetic resonance imaging revealed well-demarcated tumor of 43 mm compressing the optic nerve medially [Figure 1b and c]. However, VasoCT showed some feeders from the ophthalmic artery, which is coursing around the anterior pole of the tumor [Figure 1d].
Tumor capsule was revealed (a). We aspirated the tumor (b), which was not effective. Note the gelatinous tumor content (c). After the appropriate amount of tumor resection, optic nerve (arrow) was observed (d).
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F2
We incised the periorbita in between the superior and lateral rectus muscles and identified the tumor [Figure 2a and b]. Internal decompression using cavitron ultrasound surgical aspirator [Figure 2c] was performed followed by the tumor dissection from the adjacent tissue and resection in a piecemeal fashion. The optic nerve was identified medially displaced near the orbital apex [Figure 2d].
Hematoxylin and eosin stain showed neurofibromatosis (arrow) and schwannomatous components (double arrow). Arbitrary black line is drawn (a). Neurofilament stain showed positivity in the neurofibromatosis component (b). S100 stain showed strong positivity in the schwannomatous component (c). Original x40.
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F3
NF and schwannomatous component, respectively. Neurofilament staining showed axonal filament in NF component [Figure 3].
Postoperative imaging. Computed tomography showed the proptosis disappeared (left). Gadolinium-enhanced fat suppression T1-weighted magnetic resonance imaging showed subtotally resected tumor and well-visualized optic nerve. Note the optic nerve compression was relieved (right).
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F4
The postoperative imaging study showed completely improved proptosis and substantially reduced tumor as well as decompression of the optic nerve [Figure 4].