PMC6661383_01
Computed Tomography (CT) Angiography of the chest with arterial and venous phase revealing a well-defined soft tissue mass (arrow) within the lumen of the esophagus at its proximal third measuring around 7.8 x 5.4 x 10.5 cm in its maximal transverse, AP, and craniocaudal dimensions respectively. It shows a smooth contour with mild enhancement on the arterial phase, which increases on the venous phase images. There is no extraluminal extension. A 7 mm lymph node (arrowhead), adjacent to the esophagus in the upper mediastinum, is also noted. (A: axial; B: coronal; C: sagittal cuts).
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AP, and CC dimensions, respectively, with prominent superior mediastinal lymph nodes, the largest measuring 7 mm (Fig. 1). An upper gastrointestinal endoscopy with ultrasound showed an elevated smooth surface lesion at around 20 cm from the incisors, originating from the submucosa, abutting the muscularis mucosa, and covered by a hypervascularized mucosa (Fig. 2).
Upper gastrointestinal tract endoscopy revealing a protruding submucosal lesion at the proximal segment of the esophagus with an intact and hypervascular mucosa (A). The soft tissue mass is almost obstructing the esophageal lumen. (B) An endoscopic ultrasound image of the well-defined mass almost concealing the esophageal lumen.
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AP, and CC dimensions, respectively, with prominent superior mediastinal lymph nodes, the largest measuring 7 mm (Fig. 1). An upper gastrointestinal endoscopy with ultrasound showed an elevated smooth surface lesion at around 20 cm from the incisors, originating from the submucosa, abutting the muscularis mucosa, and covered by a hypervascularized mucosa (Fig. 2).
Intra-operative image showing the dilated residual proximal portion of the esophagus after complete resection of the schwannoma (arrow). Using this portion, a primary esophagoplasty closure was performed without compromising the esophageal caliber. Note the nasogastric tube within the lumen of the esophagus.
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The 8 cm width allowed the reconstruction of the 15 cm longitudinal defect of the esophagus without undue narrowing of the lumen (Fig. 3).
Illustrative drawing showing the longitudinal defect closure of the esophagus in two layers, using running 3-0 vicryl suture for the innermost mucosa and 3-0 Polydioxanone (PDS) for the muscular layer. The right lung, chest wall, and ligated azygous vein were shown for orientation purposes.
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The esophagus was closed in two layers using a 3-0 vicryl running suture for the mucosa and 3-0 Polydioxanone (PDS) for the muscular layers. (Fig. 4).
Gross specimen of the esophageal schwannoma after resection, measuring around 10 cm in longest dimension.
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Grossly, a 9.5*7.0*3.0 cm firm mass, covered with normal mucosa having a focal ulceration, was identified (Fig. 5).
Histopathological examination using Hematoxylin and eosin stain of the specimen revealing bundles of spindle cells originating from the submucosal layer of the esophagus (A; x40) with the presence of lymphoid infiltrates and dilated angulated vessels at the edge of the tumor (B; x100). (C; x200) There is presence of ancient changes characterized by large atypical dark nuclei, which represent a peculiar component of schwannomas. (D) Immunostaining performed on formalin-fixed paraffin-embedded sections showing diffusely positive S-100 proteins. S-100 proteins are normally present in cells derived from the neural crest such as Schwann cells and others. Other immunostains including CD34, Desmin and CD117 were negative. Ki-67 labeling index was < 2%.
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Microscopically, the tumor was composed of compact bundles of spindle-shaped cells, arranged in a fasciculated and disarrayed architecture, and nuclei arranged in a palisading pattern (Fig. 6 A-C). Immunohistochemistry revealed a diffuse positivity for S-100 (Fig. 6D), focally positive SMA, and negative staining for CD34, CD117, and Desmin, respectively.