case_images: PMC11747880_01
case_images: Computed tomography (CT) scan and 18F-fluorodeoxyglucose positron emission tomography (18F-FDG/PET) CT of a, b) the submandibular glands and c, d) at the level of pre-carina region.
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case_images: PMC11747880_01_EDU-0125-2024.01.jpg
case_images: F1
case_images: The CT scan revealed an enlarged bilateral submandibular gland (figure 1a, arrow) with hypermetabolic signal (highest maximum standardised uptake value (SUVmax) 6.82) on 18F-FDG/PET CT (figure 1b, arrow). The CT scan revealed an enlarged station 4R lymph node at the pre-carinal region (figure 1c, arrow) with hypermetabolic signal (highest SUVmax of 4.10) on 18F-FDG/PET CT (figure 1d, arrow), as well as bilateral pleural effusions.
case_images: Endobronchial ultrasound (EBUS) examination revealed an oval shaped, distinct lymph node at pre-carinal station 4R (a) with intermediate stiffness on elastography assessment (b).
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case_images: PMC11747880_01_EDU-0125-2024.02.jpg
case_images: F2
case_images: EBUS examination (BF-UC190F, Olympus Medical) then confirmed multiple enlarged mediastinal and hilar lymph nodes, with the largest at station 4R measuring 15x16 mm in diameter with intermediate stiffness on elastography (figure 2).
case_images: Endobronchial ultrasound (EBUS)-guided transbronchial needle aspiration (TBNA) was performed with 22G TBNA needle for three passes (arrow in a), followed by cryobiopsy placing the 1.1 mm cryoprobe into the target lymph node through the track created by TBNA needle and a high-frequency needle knife (arrow in b).
case_images: EDU-0125-2024.03.jpg
case_images: PMC11747880_01_EDU-0125-2024.03.jpg
case_images: F3
case_images: TBNA was performed with a 22G needle for a total of three passes (figure 3a). The 1.1 mm cryoprobe (ERBE Elektromedizin, Tubingen) was then placed under direct bronchoscopic vision into the target after track dilatation with a high-frequency needle knife (KD-31C-1, Olympus Medical) (figure 3b). The probe was then activated for 5 s, and the echobronchoscope-cryoprobe was swiftly removed en bloc from the patient.
case_images: a, b) Histopathological analysis of the 4R station cryobiopsy specimens (panel b, x20 magnification, haematoxylin and eosin stain). c, d) Further specific staining with IgG (panel c, x20 magnification, IgG stain) and IgG4 (panel d, x20 magnification, IgG4 stain). Scale bars: a) 1000 microm; b-d) 100 microm.
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case_images: F4
case_images: Histopathological analysis of the cryobiopsy tissue measuring 5 mm in total diameter is shown in figure 4a and b. Further specific staining with IgG and IgG4 was ordered due to our clinical suspicion of IgG4-RD (figure 4c and d). Morphological examination revealed the presence of lymphoid tissue characterised by dense lymphoplasmacytic infiltrates and increased eosinophil counts (figure 4b). Further specific staining with IgG (figure 4c) and IgG4 (figure 4d) revealed an IgG4/IgG ratio of 0.9.
case_images: Histology of initial salivary gland biopsy revealed storiform fibrosis (panel a, x10 magnification, haematoxylin and eosin (H&E) stain) and obliterative phlebitis (panel b, x20 magnification, H&E stain). Staining with IgG (panel c, x10 magnification, IgG stain) and IgG4 (panel d, x10 magnification, IgG4 stain) revealed high IgG4/IgG staining ratio. Scale bars: a, c, d) 200 microm; b) 100 microm.
case_images: EDU-0125-2024.05.jpg
case_images: PMC11747880_01_EDU-0125-2024.05.jpg
case_images: F5
case_images: Upon re-evaluation of the initial salivary gland biopsy by a thoracic pathologist, storiform fibrosis, obliterative phlebitis, and a high IgG4/IgG staining ratio were observed (figure 5).
case_images: 3-month surveillance computed tomography (CT) scan at the level of submandibular glands as well as station 4R lymph node. a, c) CT scan on presentation, b, d) 3-month surveillance CT scan.
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case_images: PMC11747880_01_EDU-0125-2024.06.jpg
case_images: F6
case_images: The 3-month surveillance scan confirmed a significant reduction in the size of the bilateral submandibular salivary glands (arrow, figure 6a (on presentation) versus figure 6b (3-month surveillance scan)). The pre-carinal mediastinal lymph node also demonstrated significant regression in size (arrow, figure 6c (on presentation) versus figure 6d (3-month surveillance scan)), with no recurrence of pleural effusion.