PMC10034102_01
(A) External image of the patient. (B) The mass was dissected under the table and was approximately 5.5cm x 2.3cm x 1cm in size. (C-H) Orbital MRI scan revealed right lacrimal gland was enlarged obviously and an analogous round mixed-signal (2.3cmx2.0cmx1.5cm) shadow in the right lateral posterior superior orbit with heterogeneous enhancement. T1-weighted imaging without contract enhancement and fat suppression (C). T2-weighted imaging with contract enhancement and fat suppression (D) and (E). T1-weighted imaging with contract enhancement and fat suppression (F-H).
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A 52-year-old man presented with a 2-month history of a mass in the right upper eyelid ( Figure 1A ). Contrast-enhanced magnetic resonance imaging (MRI) of the orbit revealed that the right lacrimal gland was significantly enlarged, and an analogous round mass in the right lateral posterior superior orbit with heterogeneous enhancement; no obvious abnormalities were found in the left eye ( Figures 1C-H ). While the retrobulbar portion was dark red and pale yellow with an ambiguous boundary, the lacrimal fossa portion was gray and oval with a clear boundary ( Figure 1B ).
Pathological images of the patient`s first operation (. (A) HE showed the tumor was mainly composed of squamous epidermoid cells, intermediate cells and mucinous cells with tumor necrosis (bottom left, x200). (B) Immunohistochemical staining CK7 (+,x400). (C) Immunohistochemical staining CK5/6 (+,x400). (D) Immunohistochemical staining p40 focal (+,x400). (E) Immunohistochemical staining p63 focal (+,x400). (F) Immunohistochemical staining CEA (+,x400). (G) Immunohistochemical staining Ki-67 (+, 30%,x400). (H) AB focal (+,x400). (I) PAS focal (+,x400).
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Subsequently, the hematoxylin-eosin (HE) revealed the tumor was primarily made up of epidermoid cells, intermediate cells and mucinous cells ( Figure 2A ). Immunohistochemical staining revealed that CK7 CK20 CK5/6 p63 p40 and CEA were positive whereas CD117 CD56 S-100 were negative, the Ki67 proliferation index was around 30%; in addition, the alcian blue staining (AB) and periodic acid-schiff stain (PAS) were positive ( Figures 2B-I ).
(A) External image of the first recurrence. (B) The mass was dissected under the table and was approximately 1.7cmx1.2cmx0.8cm in size. (C, D) Contract-enhanced MRI with STIR sequence of the orbit showed the absence of the right lacrimal gland and an oval abnormal signal (1.0cmx0.9cm) in the right superomedial orbit with peripheral rim enhancement.
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Eight months later, the patient returned with a right orbital mass ( Figure 3A ). An orbital MRI scan revealed a mass in the right superomedial orbit with peripheral rim enhancement, and no evident abnormalities in the left eye ( Figures 3C, D ). For reasons of prognosis, disfigurement, and vision loss, the patient chose tumor resection instead of orbital exenteration ( Figure 3B ).
(A) External image of the second recurrence. (B) The mass was dissected under the table and was approximately 2.8cmx2.5cmx1.3cm in size. (C-J) Contract-enhanced MRI with STIR sequence of the orbit showed two irregular mixed-signal shadows on the right superomedial orbit and the left superior orbit with mild enhancement (E, F, I, J red arrow). Bilateral frontal meninges were thickened and enhanced obviously, and part of them had liquefaction necrosis. An analogous round mass with peripheral rim enhancement was seen in the right frontal lobe, and significant edema with unclear boundaries was observed in bilateral frontal lobes and corpus callosum (C, D, G, H red arrow). Additionally, numerous irregular abnormal signals with heterogeneous enhancement were observed in bilateral ethmoid sinuses, maxillary sinuses, sphenoid sinuses, frontal sinuses, right infratemporal fossa, face, zygomatic area, and parotid region, involving some bone.
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Two months later, the patient returned again with gait instability caused by cerebral metastasis, an MRI scan revealed an analogous round mass with peripheral rim enhancement in the right frontal lobe, diffuse thick pachymeningeal enhancement along bilateral frontal lobes, and widespread edema in both frontal lobes and corpus callosum ( Figures 4C, D, G, H ). It also revealed two masses, one in the right superomedial orbit and the other in the left superior orbit, which indicated the recurrence and metastasis of an orbital tumor ( Figures 4E, F, I, J ). Two months later, due to the intense pain in the left eye, the patient returned for treatment to our department ( Figure 4A ) and had the tumor in the left orbit resected. Intraoperatively, a grayish-white neoplasm was found in the left superior orbit with the orbital roof and levator palpebrae superioris muscle involved ( Figure 4B ).
Pathological images of the patient`s fourth operation. (A) HE showed infiltrating tumor cells mainly consisted of squamous epithelial and clear cells (top left of A,x200). (B) Immunohistochemical staining CK7 (+,x400). (C) Immunohistochemical staining showed a few cells CK5/6 (+,x400). (D) Immunohistochemical staining Ber-EP4 (+,x400). (E) Immunohistochemical staining Ki-67 (+, 40%,x400). (F) Immunohistochemical staining p63(-,x400).
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The pathological diagnosis was high-grade metastatic MEC ( Figure 5 ).