PMC9634463_01
Patient's clinical appearance at the first presentation, showing right palpebral ptosis and exophthalmos. This image is published with the patient's daughter's consent.
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The physical examination revealed third-grade exophthalmos on the right eye and a firm and fixed mass on the right superior orbit on palpation (Figure 1).
Ocular ultrasonography of the right eye. Red circles show a large mass arising from the lacrimal gland, reaching the temporal lobe.
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Ultrasonography of the right eye showed the presence of a large mass arising from the lacrimal gland, extending to the temporal lobe and occupying I/II/III Benedikt's space, compatible with lymphoma (Figure 2).
Cranio-facial computed tomography scan (CT scan) showing the lesion located at the upper outer portion of right orbit, with bone erosion, muscle and pachymeningeal infiltration.
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The lesion infiltrated the right lateral rectus muscle and part of the right superior rectus muscle and caused the medial displacement of the right optic nerve, with initial signs of pachymeningeal enhancement in the right front-orbital area (Figure 3).
Coronal fusion images of 18F-FDG PET/CT scan showing 18 F-FDG uptake in the right orbit, multiple FDG-positive pulmonary nodules, bone metastasis and a lesion with a maximum diameter of 50 mm in the right kidney. 18F-FDG PET/CT, 18-fluorodeoxyglucose-positron emission tomography/computerized tomography.
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A subsequent 18-fluorodeoxyglucose-positron emission tomography/computerized tomography scan (18F-FDG PET/CT scan) showed a lesion on the right orbit measuring approximately 25x38 mm, with diffuse tracer uptake in the right orbit (SUVmax 5.5), lateral and posterior wall erosion of orbit and with extra-orbital extension, subcentimetric multiple lung nodules, the greater located on inferior lobe (SUVmax 3.9), a hyperdense lesion enclosing hypodense necrotic areas in the lower third of right kidney with a maximum diameter of 50 mm (SUVmax 12.3), and disseminated bone involvement with mixed lytic and sclerotic bone metastases (Figure 4).
Hematoxylin and eosin staining. Magnification x2.
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Immunohistochemistry showed positivity for paired-box gene 8 (PAX8), cytokeratin 19 (CK 19) and renal cell carcinoma marker (RCC-Ma) and negativity for S100, thyroid transcription factor-1 (TTF1), human melanoma black-45 (HMB45), p63, cytokeratin 7 (CK 7), placental alkaline phosphatase (PLAP) and GATA binding protein 3 (GATA 3) (Figures 5-7).
Immunohistochemical staining for RCC-Ma. Magnification x2.8. RCC-Ma, renal cell carcinoma marker.
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Immunohistochemistry showed positivity for paired-box gene 8 (PAX8), cytokeratin 19 (CK 19) and renal cell carcinoma marker (RCC-Ma) and negativity for S100, thyroid transcription factor-1 (TTF1), human melanoma black-45 (HMB45), p63, cytokeratin 7 (CK 7), placental alkaline phosphatase (PLAP) and GATA binding protein 3 (GATA 3) (Figures 5-7).
Immunohistochemical staining for PAX8. Magnification x2. PAX8, paired-box gene 8.
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Immunohistochemistry showed positivity for paired-box gene 8 (PAX8), cytokeratin 19 (CK 19) and renal cell carcinoma marker (RCC-Ma) and negativity for S100, thyroid transcription factor-1 (TTF1), human melanoma black-45 (HMB45), p63, cytokeratin 7 (CK 7), placental alkaline phosphatase (PLAP) and GATA binding protein 3 (GATA 3) (Figures 5-7).