PMC7652604_01
Gross appearance. The preoperative tongue (a), the operation design (b), the intraoperative status (c), the resected tongue (d), and the postoperative tongue (e).
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fig1
She did not complain of oral discomfort and underwent surgery to prevent problems with articulation (Figure 1(a)). The tongue was partially resected using the keyhole-shaped technique (Figures 1(b)-1(d)). There were no postoperative complications or recurrence of macroglossia for 2 years (Figure 1(e)).
PMC7652604_02
The autopsy tongue specimen of a 1-year and 8-month-old girl (control). The sagittal section has been shown. The subepithelial tissues in the posterior side are composed of muscle fibers and minor salivary glands (arrowhead) (a) (loupe). Muscle fibers are composed of longitudinal and cross section regularly, and interstitial spaces between muscle fibers can be seen on both sides (b and c) (100x, scale bar: 200 mum). IGF2 protein expression in the muscle fibers does not differ between the anterior (d) (200x) and posterior sides (e) (200x).
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fig2
As a control, we examined the autopsied tongue of a 1-year and 8-month-old girl who did not have a growth disorder (Figures 2 and 3). At low magnification, the hematoxylin-and-eosin-stained patient specimen exhibited a dense subepithelial eosinophilic area in the posterior side (Figures 2(a) and 3(a)). Subepithelial minor salivary glands were scarce in the patient tongue but easily detectable in the control (Figures 2(a) and 3(a)). Loose interstitial spaces were observed on both sides of the control specimen, but only on the anterior side of the patient specimen (Figures 2(b), 2(c), and 3(b)). In the control specimen, IGF2 protein was more highly expressed in the longitudinal sections compared with the transverse sections, and the staining pattern or intensity did not differ between the anterior and the posterior sides of the tongue (Figures 2(d) and 2(e)).
Histology of macroglossia associated with Beckwith-Wiedemann syndrome in the present case. The sagittal section shows an enlarged subepithelial eosinophilic area that is more prominent on the posterior side. Few salivary glands can be seen (arrowhead) (a) (loupe). On the anterior side, loose interstitial spaces are present between the skeletal muscle bundles (b) (100x, scale bar: 200 mum). On the posterior side, several skeletal muscle bundles are closely arranged in a storiform or fascicular pattern (c) (100x, scale bar: 200 mum). Variable and heterogeneous IGF2 protein levels are seen on the anterior side (d) (200x) compared to the more intense and homogenous staining on the posterior side (e) (200x).
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fig3
As a control, we examined the autopsied tongue of a 1-year and 8-month-old girl who did not have a growth disorder (Figures 2 and 3). The posterior side (body of the tongue) was harder than the anterior (the anterior of the tongue; Figure 3(a)). The dense subepithelial eosinophilic area consisted of longitudinal sections of skeletal muscle bundles that were prominent on the posterior side and tightly packed in a storiform or fascicular pattern (Figure 3(c)). However, in the patient specimen, IGF2 protein was detected to similar extent in both sections, and more intense staining was observed on the posterior side compared with the anterior (Figures 3(d) and 3(e)).