case_images: PMC8328685_01
case_images: CT maxillofacial with IV contrast showing opacified ethmoid sinuses with bone destruction of the right lamina papyracea with soft-tissue extension into the right orbit. (a) Coronal view; (b) axial view.
case_images: CRIHEM2021-9972694.001.jpg
case_images: PMC8328685_01_CRIHEM2021-9972694.001.jpg
case_images: fig1
case_images: Also shown was destruction and soft-tissue swelling that extends along the medial border of the right orbit (Figure 1).
case_images: Timeline highlighting diagnosis, treatment schedule, disease course, and findings.
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case_images: PMC8328685_01_CRIHEM2021-9972694.002.jpg
case_images: fig2
case_images: He was started on a modified SMILE (m-SMILE) regimen consistent of methotrexate 2 g/m2 given on day 1, etoposide 100 mg/m2, ifosfamide 1500 mg/m2, dexamethasone 40 mg/d given on days 2 to 4, and pegasparagase 2000 U/m2 given on day 8 of a 28-day cycle (Figure 2).
case_images: PET/CT scan highlighting the maxillofacial structures. (a) After 2 cycles of mSMILE, a single 1.1 cm focus of increased FDG uptake was seen along the right sinonasal cavity, SUV max 9.3. (b) Restaging PET/CT scan after 6 cycles of mSMILE showing mild residual uptake in the right maxillary sinus, SUV max 4.2.
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case_images: PMC8328685_01_CRIHEM2021-9972694.003.jpg
case_images: fig3
case_images: He completed his second cycle of m-SMILE, and a follow-up positron emission tomography-computed tomography (PET/CT) scan showed good response (Figure 3(a)). A restaging PET/CT scan showed good response with faint residual disease in the paranasal sinuses and sinonasal cavity (Figure 3(b)).
case_images: MRI brain with IV contrast showing several scattered bilateral foci of increased T2/FLAIR signal in the bifrontal regions. Also noted, as shown by the blue arrows, was a diffuse swelling of the gyri involving bilateral frontal and anterior parietal lobes.
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case_images: fig4
case_images: Subtle enhancement in the sulci of the high frontoparietal region, particularly at the central sulcus, was noted (Figures 4 and 5 ).
case_images: MRI brain with IV contrast suggesting sulcal enhancement of the gyrus representing underlying leptomeningeal disease.
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case_images: PMC8328685_01_CRIHEM2021-9972694.005.jpg
case_images: fig5
case_images: Subtle enhancement in the sulci of the high frontoparietal region, particularly at the central sulcus, was noted (Figures 4 and 5 ).