Paraganglioma with high-grade intraepithelial neoplasia in the oesophagus treated by endoscopic submucosal dissection.

Abstract

A 37-year-old woman was hospitalised with a 4-month history of retrosternal pain. Gastroscopy revealed a protrusion lesion of approximately 0.6 cm in diameter in the left wall of the oesophagus, approximately 27 cm from the incisor (Fig. 1). Consequently, one biopsy specimen was taken from the oesophagus. Based on the pathology and immunohistochemistry findings, the patient was initially diagnosed with paraganglioma with high-grade squamous intraepithelial neoplasia. The patient underwent endoscopic submucosal dissection 12 days after the biopsy. The protrusion lesion was again observed under gastroscopy, with visible scarring on the surface (Fig. 2A). A hypoechoic and heterogeneous mass of about 7.1 mm × 2.0 mm in the muscularis mucosae layer was detected on endoscopic ultrasonography (Fig. 2B). The mass was excised after submucosal injection of adrenaline, melphalan, and glycerol fructose injection (Fig. 2C). The postoperative gross specimen was a mucosal tissue measuring 2 cm × 1.8 cm × 0.2 cm, with a bulging mass underneath (Fig. 2D). The results of immunohistochemical analysis were positive for CD56, Syn, CK8/18, LCK, and S-100 and negative for SSTR2 (Fig. 3D, E). Expression of wild-type P53, focal positivity of CgA, and weak positivity of NSE were noted. The Ki-67 labelling index was 1–2%. The diagnosis of paraganglioma with high-grade squamous intraepithelial neoplasia was confirmed based on the histopathology findings (Fig. 3A, B, C). The patient recovered well during the follow-up. Fig. 2(A) The protrusion lesion was found again under gastroscopy, with visible scarring on the surface. (B) Endoscopic ultrasonography detected a hypoechoic and heterogeneous mass of about 7.1 mm × 2.0 mm in the muscularis mucosae layer. (C) Adrenaline, melphalan and glycerol fructose injection was injected in the submucosa after the lesion was marked with a dual knife. (D) Gross specimen after endoscopic submucosal dissection. View Large Image Figure Viewer Download Hi-res image Fig. 3Microscopic examination. (A) Significant cytological atypia in squamous epithelium with continuous basement membrane, and arrangement of the cells in nests (“Zellballen”) in muscularis mucosae. (HE stain × 4) (B) The tumour was composed of chief cells and sustentacular cells. (HE stain × 10) (C) The chief cell is polygonal with a fine granular cytoplasm of acidophilia as well as basophilia. The nucleus is round to elliptical. The sustentacular cells of spindle-shape are distributed around the tumour cell nests or interdigitated amongst the sustentacular cells. (HE stain × 40) (D) positivity of S-100 protein (× 10). (E) positivity of Syn. (× 10). View Large Image Figure Viewer Download Hi-res image