PMC9035948_01
Axial (a-c), coronal (c, d), and sagittal (b, d) CT showing a well-defined, spherical solid mass in the upper thoracic esophagus presenting with mild uniform enhancement, which caused proximal esophagus dilatation.
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F1
Chest computed tomography (CT) showed a spherical soft tissue mass in the upper thoracic esophagus, with obvious narrowing of the lumen (Fig. 1).
Endoscopic ultrasonography of the esophagus before operation. a Hemispherical protruding lesion in the upper thoracic esophagus with an intact mucosal surface, presented with the formation of mucosal bridges. b Lesion originated from the esophageal muscularis propria, and the adventitia is smooth and continuous.
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F2
Flexible esophagoscopy confirmed a large polypoid mass 18 cm away from the incisor teeth, completely obstructing the esophagus within the intact mucosal surface, presenting with formation of mucosal bridges (Fig. 2a). Endoscopic ultrasonography of esophagus showed that the lesion arose from the esophageal muscularis propria with a smooth and continuous adventitia (Fig. 2b).
The pathological and FISH results of pPNET of the upper thoracic esophagus. a H&E stain showed a uniform population of small round blue cells with markedly vesicular nuclei and finely dispersed chromatin, accompanied by some remarkable hemorrhage. b Homer-Wright rosettes (arrow) were seen on H&E. c, d Immunohistochemistry of CD99 (c) showed a diffuse strong membrane staining, but synaptophysin (d) was negative in the tumor cells. e FISH by the EWSR1/FLI1 Fusion Translocation t(11;22) Probe illustrating the EWSR1/FLI1 gene rearrangement associated with t(11;22)(q24;q12). Note: green signal (e) is GSP EWSR1, and red signal (e) is GSP FLI1 (original magnification x20 (a), x40 (b-d)). H&E, hematoxylin and eosin.
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F3
Histologically, the tumor consisted of a markedly cellular tissue with a trabecular growth pattern accompanied by hemorrhage on hematoxylin and eosin staining (Fig. 3a). In addition, the classical Homer-Wright rosettes were also seen (Fig. 3b). Tumor cells had small, uniform, mildly atypical nuclei with granular chromatin and small amounts of cytoplasm (Fig. 3a, b). Co., Ltd., Fuzhou, China). CD99 (clone O13; Maixin) (Fig. 3c) and P63 (clone MX 013; Maixin) were positive in the tumor cells. D5; Maixin) (Fig. 3d). Translocation t(11;22) Probe (Guangzhou LBP Medicine Science & Technology Co., Ltd., Guangzhou, China), and two hundred interphase cells were examined. The patient's sample was positive for EWS/FLI1 transcripts associated with t(11;22)(q24;q12) (Fig. 3e), which signal modes are as follows: 1 G1R1F 37.0%, 2 G1R1F 10.0%, 2 G2R1F 4.0%, 1 G1F 10.0%, 2 G1F 10.0%, 1R1F 7.5%, 1R2F 5.0%, 1 G2F 7.5%, and 1 G1R2F 9.0%.
Computed dosimetric reconstruction images used for IMRT. a-c PGTVtb (red color wash image) and PTV (green color wash image) were administered 56 Gy (shown in blue isodose line) and 50.4 Gy (shown in red isodose line), respectively. Note: GTVtb refers to gross tumor volume of TB, and CTV refers to GTVtb with a radial margin of 0.5-1.0 cm and a longitudinal margin of 3 cm; CTV and GTVtb with a margin of 0.5 cm in three dimensions formed the PTV and PGTVtb. IMRT, intensity-modulated radiation therapy; PGTVtb, planning gross tumor volume of the tumor bed; PTV, planning target volume.
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F4
Chest radiation was delivered using intensity-modulated radiation therapy (Fig. 4a-c) as follows: the planning gross tumor volume of the tumor bed (TB) (PGTVtb) and planning target volume (PTV) were administered using 56 and 50.4 Gy in 28 daily fractions of 1.8-2.0 Gy, respectively.