PMC8208880_01
Case 1. (a, b) Clinical photograph shows alteration of the external ocular movements with limitation to supraduction and abduction.
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In the RE, she also presented alteration of the external ocular movements with limitation to supraduction and abduction (Figures 1(a) and 1(b)), proptosis of 9 mm (Figure 2), lagophthalmos of 3-4 mm, and a mass in the upper orbital region.
Case 1. Clinical photograph shows proptosis of 9 mm in the right eye.
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In the RE, she also presented alteration of the external ocular movements with limitation to supraduction and abduction (Figures 1(a) and 1(b)), proptosis of 9 mm (Figure 2), lagophthalmos of 3-4 mm, and a mass in the upper orbital region.
Case 1. Orbital echography shows a superior extraconal mass with high density and irregular pattern.
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Orbital echography showed an extraconal superior mass with high density and an irregular pattern (Figure 3).
Case 1. (a) Magnetic resonance imaging (MRI) in coronal image showing T2-weighted extraconal tumor based on superior and central orbital, which measures 3.7 cm in the greatest axis and hyperintensity image. (b) T1 weighted with hypointensity image.
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In the magnetic resonance imaging (MRI), an extraconal, superior, and central orbital mass was observed; it measured 3.7 x 3.5 x 1.7 cm and extended up to the posterior third of the orbit, with hyperintensity on T2-weighted scans and hypointensity in T1-weighted scans (Figures 4(a) and 4(b)), compatible with cavernous angioma or meningioma.
Case 1. Gross examination demonstrated a pink, indurated, well-circumscribed, and encapsulated appearance.
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An upper orbitotomy was performed through the upper lid crease with dissection and removal of a compact, depressible pink lesion, which is well defined and without nutrient vascularization (Figure 5).
Case 1. (a, b) Microscopic appearance of the tumor, composed of a proliferation of spindle cells arranged in an irregular manner with a focal storiform pattern, bland cytology, and vascular proliferation with a hemangiopericytoma-like growth pattern (arrow in (b)). (H&E, x40 and x100 magnification.) Positivity of the tumor for (c) BCL2 and (d) CD34. (Bar is indicated in mum, down and to the right).
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Immunohistochemistry was positive for CD34, CD99, BCL2, vimentin, and myoglobin (Figures 6(a)-6(d)).
PMC8208880_02
Case 2. Clinical photograph shows proptosis of 4 mm in the right eye.
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At the last visit, the patient was assessed by ophthalmology because of a 4 mm exophthalmos (Figure 7), with inferior and external displacement of the RE, and limitation to adduction and supraduction.
Case 2. (a) MRI in axial view showing the initial large, well-defined round mass over the superior and posterior orbits. Axial computed tomography (CT) imaging showing (b) the third recurrence which includes ethmoidal invasion 3 years later (c) and a fifth recurrence 5 years later.
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Magnetic resonance imaging (Figure 8(a)) and computed tomography (CT) imaging (Figure 8(b)) with contrast showed a 2.2 cm mass that captured contrast; it was located in the right ethmoid sinus with medial invasion into the orbit with infiltration of the medial rectus muscle and minimal posterior extension to the sphenoid (Figure 8(c)).
Case 2. (a) Microscopically, the tumor reveals fibroblastic proliferation with hypo- and hypercellular areas alternating with each other and bands of sclerotic collagen fiber networks (arrow points at the more hypocellular area) (H&E, x40 magnification). (b) Higher magnification demonstrates the cellular phase of the tumor with tightly packed fusiform cells arranged around a ramifying vascular network (H&E, x100 magnification). Positivity of the tumor for (c) CD34 and (d) CD99. Bar is indicated in mum, down and to the right.
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There was no significant cellular atypia and mitosis was 1-4/10 HPF (Figures 9(a) and 9(b)). On immunohistochemistry, the tumor cells had typically strong and diffuse positivity for vimentin and BCL2, focal positivity for CD34, and weak diffuse positivity for CD99 but negativity for cytokeratins AE1-AE3, actin HHF35, actin 1A4, calponin, caldesmon, desmin, S-100 protein, and collagen IV (Figures 9(c) and 9(d)).
PMC8208880_03
Case 3. (a) CT imaging showing a well-defined rounded lesion, in the left orbital apex. (b) Coronal view. (c) Sagittal view.
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It was rounded and well defined with a diameter of 16 mm (Figures 10(a)-10(c)).
Case 4. (a) Photograph of surgical exenteration. Temporal muscle fills the posterior part of the right orbit. (b) Axial view MRI postexenteration.
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Due to the aggressive nature of the tumor, exenteration and reconstruction of the left orbit with a temporal muscle flap were performed (Figures 11(a) and 11(b)).
PMC8208880_04
Case 4. (a) CT imaging with a great lesion, hyperdense morphology appears in the right intraconal region. The lesion imprints on the back of the globe, as well as the optic nerve and internal rectus muscle, with close contact with the external rectus muscle. (b, c) MR imaging, coronal view, presents lesion isodensity in T1- and T2-weighted sequences.
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Due to the previous neurosurgery, there was residual frontal cerebral lobe basal hypodensity, as well as hypodensities in white matter of both convexities in the frontal and temporal vertebral lobes (Figure 12(a)). In the cranial study, areas of signal hyperintensity were evident in T2 and FLAIR sequences in the bilateral frontobasal white substance (Figures 12(b) and 12(c)), bilateral temporobasal substance, and both internal capsules, all related to residual ischemic events/gliosis.
Case 4. (a) It shows the histological appearance of a moderately fibroblastic neoplasia with a mostly storiform pattern. The tumor is composed of bland and uniform oval to spindle cells dispersed along thin parallel collagen bands with hypocellular areas (H&E, x100 magnification). (b) Hypercellular areas (H&E, x200 magnification). Positivity for (c) CD34 and (d) BCL2. Bar is indicated in mum, down and to the right.
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There were also numerous vascular clefts "in deer antler." Necrosis or hemorrhage foci were not observed (Figures 13(a) and 13(b)). There was negativity for CD31 except in the vascular endothelium (Figures 13(c) and 13(d)).