PMC5950185_01
Preoperative facial photograph. Frontal view of the patient before operation, showing fullness of left inferolateral orbital and periorbital region with slightly upward displacement of the lateral canthus
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Fig1
There was fullness in the inferolateral orbital and periorbital areas (Fig. 1) and a firm, regular mass was palpable in the region. Occasional mitotic figures (1 per 20 high power fields) were seen.
Preoperative imaging. CT scan reveals an isodense intraosseous lesion in the zygomatic bone in axial (a) and coronal sections (b). In orbital MRI, the mass appears heterogenous in T2-weighted images (c) and shows remarkable contrast enhancement after gadolinium injection (d)
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Fig2
Orbital CT scan revealed a well-defined round isodense intraosseous lesion in the lowermost portion of the lateral orbital wall, expanding the bone and protruding anteriorly and medially (Fig. 2a and b). MRI showed the mass to be heterogenous and strongly enhancing with contrast medium (Fig. 2c and d).
Pathologic findings. Histopathologically (a and b), the tumor is composed of several slit-like vascular channels with occasional stag-horn appearing structures, surrounded by numerous spindle cells (H&E stain, low and high magnification, respectively). Immunohistochemistry shows positive staining for CD31 and CD34 in endothelial cells of blood vessels (c) and also positive staining for smooth muscle actin (SMA) in spindle cells (d)
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Fig3
Histopathologic examination revealed the mass to be composed of several slit-like vascular channels, with occasional stag-horn appearing vessels, surrounded by elongated bland-looking spindle cells (Fig. 3a and b). Immunohistochemistry (IHC) staining results were positive for CD34 and CD31 in endothelial cells (Fig. 3c) and for smooth muscle actin (SMA) in spindle cells (Fig. 3d), but negative for CD99, S100, B-cell lymphoma 2 (bcl-2) and desmin, suggesting the presence of HPC.
Postoperative facial photograph. Frontal view of the patient 5 years after the surgery, showing complete resolution of inferolateral orbital fullness
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Fig4
The postoperative course was uneventful, with the patient doing well without clinical or radiologic signs of recurrence after 5 years follow up (Figs. 4 and 5a, b).
Postoperative imaging. Orbital CT scan reveals mild secondary hyperostosis in the previous location of the mass, with reasonable healing and no remnant of the original tumor (a: axial section, b: coronal section)
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Fig5
The postoperative course was uneventful, with the patient doing well without clinical or radiologic signs of recurrence after 5 years follow up (Figs. 4 and 5a, b).