A Granular Cell Tumor Arising in a Patient with Long-segment Barrett's Esophagus

Abstract

Esophageal cell tumors are rare. Esophagogastroduodenoscopy performed on a 48-year-old woman revealed an elevated esophageal lesion and the presence of long-segment Barrett's esophagus. Endoscopic ultrasonography showed a 15 mm homogeneous hypoechoic tumor extending from the lamina propria mucosa to the submucosa. Pathological examination of the biopsy tissue revealed a sheet-like cluster of histiocytoid cells with an abundant eosinophilic granular cytoplasm. Immunohistochemical examination revealed S-100(+) and CD68(+), thus suggesting the diagnosis of a granular cell tumor. The tumor was resected by endoscopic submucosal dissection. Pathologically, the background mucosa was Barrett's mucosa. This is the first reported case of an esophageal granular cell tumor in long-segment Barrett's esophagus.

Keywords: endoscopic submucosal dissection; granular cell tumor; long-segment Barrett's esophagus.

Figure 1.

Findings of esophagogastroduodenoscopy. (A) A submucosal tumor with a central depression in the middle esophagus is seen. The surface of the tumor is covered by a glandular epithelium. (B) Long-segment Barrett’s esophagus is classified as C3M5. (C) Narrow band imaging endoscopy shows an elevated lesion overlaid by a non-neoplastic mucosa with enlargement of mucosal pattern and dilation of mucosal microvessels. (D) The homogeneous hypoechoic tumor is localized from the lamina propria to the submucosa by miniature probe endoscopic ultrasonography (mEUS, 20 MHz).

Figure 2.

(A) Histopathology of biopsy specimens reveals a sheet-like cluster of histiocytoid cells with abundant eosinophilic granular cytoplasm and small oval nuclei (Hematoxylin and Eosin staining, ×400). The histiocytoid cells are positive for S-100 (B) and CD68 (C) (immunohistochemistry, ×400).

Figure 3.

(A) Macroscopic view of the fresh specimen resected by endoscopic submucosal dissection, showing a lightly yellowish elevated lesion with a central depression, surrounded by a brownish mucosa. (B) In a whole mount histologic view, the lesion is localized from the deep lamina propria mucosa to the superficial submucosa. (C) In a high power view, the lesion reveals a sheet-like cluster of histiocytoid cells with abundant eosinophilic granular cytoplasm and small oval nuclei, covered by regenerative intestinalized cardiac gland-type mucosa with variable-sized congestive capillaries beneath the surface epithelium [Hematoxylin and Eosin (H&E) staining, ×200]. (D) The surrounding mucosa shows irregularly shaped intestinalized pits with deep cardiac-type glands, double muscularis mucosae (thin arrows) with palisade vessels between them (arrowheads), and ducts of esophageal glands (thick arrow), which thus made it possible to diagnose Barrett’s mucosa (H&E staining, ×100).