PMC8502888_01
(a) Computerized tomography shows a solid, non-enhanced nodule in the right internal jugular vein (RJV) (red arrow: nodule, green arrow: RJV). (b) Ultrasound shows the nodule arising from the RJV without extravascular invasion (red arrow: nodule, green arrow: RJV). (c) Ultrasound shows the nodule led to severe RJV stenosis (red arrow: nodule, green arrow: RJV, red triangle: right internal carotid artery). (d) Doppler ultrasound detected the blood flow signals in the nodule.
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F1
A computed tomography angiography (CTA) demonstrated the right jugular vein filling defect at the level of the cricoid cartilage-thyroid plane (Figure 1a). The grayscale ultrasound and color Doppler flow imaging showed the right internal jugular vein had a hypoechoic mass about 10 x 11 x 11 mm with a clear boundary, and the mass had a 3.9-millimeter-wide base located in the posterior wall of the internal jugular vein with a linear blood flow signal in this mass (Figures 1b,c). The pulse Doppler ultrasonography detected arterial spectrum in the mass with the peak systolic blood flow velocity: 24 cm/s, the end-diastolic blood flow velocity: 6.87 cm/s, and the resistance index: 0.71 (Figure 1d). The ultrasound indicated neoplastic lesions originating from the internal jugular vein (Figures 1b,c).
(a) The nodule arose from the RJV. (b) Grossly, the mass was pink hyaline, oval, and well-demarcated, measuring ~5 mm in diameter. (c) Extravascular tissues were not affected. (d) Due to the proximal of RJV compensatory dilation, local stenosis was observed in the RJV after reconstruction.
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F2
The specimen excised was a 13 x 10 x 18 mm mass without extravascular invasion and involvement of any surrounding structures (Figure 2).
(a) There is a proliferation of spindle cells arranged in intersecting fascicles (hematoxylin and eosin, original magnification 40x) (b). The immunohistochemical stains demonstrated elastin stains was negative (original magnification 20x) (c). The spindle cells are diffusely positive for smooth muscle actin (SMA, original magnification 20x) (d). FISH showing rearrangement of the USP6 locus (separation of green and red signals).
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F3
Fluorescent in situ hybridization (FISH) showing rearrangement of the USP6 locus (separation of green and red signals) and excluding gene amplification of the MDM2 (Figure 3).