PMC6243357_01
(a) Fluid-attenuated inversion-recovery and (b) T1 weighted contrast-enhanced MRI showing a 1.4-cm irregular ring-like enhancing lesion within the superior left frontal lobe at the grey-white junction (T1 and T2 isointense with surrounding vasogenic oedema) and a 1.3-cm lesion with a similar appearance in the region of the frontal operculum, also at the grey-white interface.
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fig1
Contrast-enhanced MRI of the brain revealed two enhancing brain lesions (Figure 1), and the patient was referred for 18F-fludeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, which showed innumerable 18F-FDG-avid lung lesions with a maximum standardized uptake value of 12.1 (Figures 2 and 3), which were confirmed to be Grade 3/3 lymphomatoid granulomatosis (LYG) on lung wedge biopsy (Figure 4) [certain nodules showed >100 Epstein-Barr virus (EBV)-encoded RNA-positive cells per high power field, Figure 5].
18F-fludeoxyglucose positron emission tomography/CT scan (Biograph, Siemens Medical Solutions, Knoxville, TN) maximum intensity projection (a) anterior and (b) left lateral images showing innumerable 18F-fludeoxyglucose-avid lung lesions (SUVmax 12.1) and a distal oesophageal lesion (SUVmax 8.5) [arrow in (a)]. SUVmax, maximum standardized uptake value.
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fig2
Contrast-enhanced MRI of the brain revealed two enhancing brain lesions (Figure 1), and the patient was referred for 18F-fludeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, which showed innumerable 18F-FDG-avid lung lesions with a maximum standardized uptake value of 12.1 (Figures 2 and 3), which were confirmed to be Grade 3/3 lymphomatoid granulomatosis (LYG) on lung wedge biopsy (Figure 4) [certain nodules showed >100 Epstein-Barr virus (EBV)-encoded RNA-positive cells per high power field, Figure 5].
Transaxial (a) positron emission tomography/CT fusion, (b) positron emission tomography and (c) CT images showing the innumerable 18F-fludeoxyglucose-avid lung nodules, which were irregular but well defined on CT images, and a distal oesophageal focus of intense 18F-fludeoxyglucose uptake [arrow in (b)].
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fig3
Contrast-enhanced MRI of the brain revealed two enhancing brain lesions (Figure 1), and the patient was referred for 18F-fludeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, which showed innumerable 18F-FDG-avid lung lesions with a maximum standardized uptake value of 12.1 (Figures 2 and 3), which were confirmed to be Grade 3/3 lymphomatoid granulomatosis (LYG) on lung wedge biopsy (Figure 4) [certain nodules showed >100 Epstein-Barr virus (EBV)-encoded RNA-positive cells per high power field, Figure 5].
Lung wedge biopsy. (a) Low power (OM 20x) haematoxylin and eosin stain of a lung nodule; (b) low power (OM 20x) periodic acid-Schiff stain showing angiocentric pattern and areas of necrosis and granulomas, consistent with Grade 3 lymphomatoid granulomatosis; (c) high power (OM 500x) showing numerous large atypical cells (arrows); (d) (OM 500x) showing an atypical cell (small arrow) and necrosis with palisaded granulomatous histiocytes (large arrow). OM, original magnification.
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fig4
Contrast-enhanced MRI of the brain revealed two enhancing brain lesions (Figure 1), and the patient was referred for 18F-fludeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, which showed innumerable 18F-FDG-avid lung lesions with a maximum standardized uptake value of 12.1 (Figures 2 and 3), which were confirmed to be Grade 3/3 lymphomatoid granulomatosis (LYG) on lung wedge biopsy (Figure 4) [certain nodules showed >100 Epstein-Barr virus (EBV)-encoded RNA-positive cells per high power field, Figure 5].
Positive Epstein-Barr virus-encoded RNA staining (OM 500x); (b) positive CD30 staining (OM 500x) and (c) positive PAX5 staining (OM 400x). OM, original magnification; PAX5, paired box 5.
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fig5
Contrast-enhanced MRI of the brain revealed two enhancing brain lesions (Figure 1), and the patient was referred for 18F-fludeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) imaging, which showed innumerable 18F-FDG-avid lung lesions with a maximum standardized uptake value of 12.1 (Figures 2 and 3), which were confirmed to be Grade 3/3 lymphomatoid granulomatosis (LYG) on lung wedge biopsy (Figure 4) [certain nodules showed >100 Epstein-Barr virus (EBV)-encoded RNA-positive cells per high power field, Figure 5].
A follow-up 18F-fludeoxyglucose positron emission tomography/CT performed 4 weeks after completion of the fourth cycle of rituximab. (a) Anterior and (b) lateral maximum intensity projection images showing complete metabolic resolution of all of the lung nodules as well as the Epstein-Barr virus-positive oesophageal ulcer.
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fig6
A follow-up PET/CT scan performed 4 weeks after completion of rituximab showed complete metabolic resolution of LYG lung lesions as well as the EBV oesophageal ulcer (Figure 6), and a follow-up MRI revealed complete resolution of the brain lesions (Figure 7).
(a) Fluid-attenuated inversion-recovery and (b) T2 weighted images. A follow-up MRI performed at the same time as follow-up positron emission tomography/CT scan showed that the previously seen left superior frontal and left inferior frontal lesions had resolved.
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fig7
A follow-up PET/CT scan performed 4 weeks after completion of rituximab showed complete metabolic resolution of LYG lung lesions as well as the EBV oesophageal ulcer (Figure 6), and a follow-up MRI revealed complete resolution of the brain lesions (Figure 7).
A follow-up 18F-fludeoxyglucose positron emission tomography/CT scan performed 12 months after the completion of rituximab therapy. (a) Anterior and (b) lateral maximum intensity projection images do not show any 18F-fludeoxyglucose-avid lung lesions, confirming disease remission.
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fig8
Surveillance PET/CT (Figure 8) and MRI (Figure 9) studies performed 12 months later confirmed disease remission.
(a) Fluid-attenuated inversion-recovery and (b) T2 weighted images. A follow-up MRI performed 12 months after completion of rituximab therapy did not show any brain lesions.
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fig9
Surveillance PET/CT (Figure 8) and MRI (Figure 9) studies performed 12 months later confirmed disease remission.