Rare Primary Esophageal Paget’s Disease Diagnosed on a Large Bloc Specimen Obtained by Endoscopic Mucosal Resection

Abstract

Primary esophageal Paget's disease is rare. Only a few case reports have described the intraepithelial papillary capillary loop (IPCL) pattern obtained by magnified Narrow Band Imaging (M-NBI) endoscopy in this rare pathology. This report highlights the usefulness of M-NBI and the successful diagnosis using a large bloc specimen obtained by endoscopic mucosal resection with the cap method (EMR-c). A 53-year-old man was referred to endoscopic examination for dysphagia. The endoscopic image revealed a ring-shaped scarring of the esophagus suggestive for eosinophilic esophagitis. The IPCL pattern by M-NBI endoscopy showed an inflammatory pattern, and the entire epithelium of the esophagus was not stained by Lugol iodine spraying. Based on six biopsies randomly performed, a poorly differentiated adenocarcinoma was diagnosed. Since the M-NBI pattern and the histology were completely different, EMR-c was performed to obtain large bloc specimens for a more detailed diagnosis. The pathological findings revealed extensive Paget's cells infiltration into the epithelium and multifocal invasion from the mucosa to the submucosal layer with adenocarcinoma. In conclusion, a large bloc specimen by EMR-c might be more useful than a small biopsy for an accurate diagnosis of the rare esophageal Paget's disease.

Histopathological landscape of rare oesophageal neoplasms

“…Background: Paget’s disease is thought to be a peculiar type of epidermotropic adenocarcinoma of mammary and extramammary tissues[ 80 ]. Extramammary Paget’s disease is most commonly seen in the vulva and anus, while an oesophageal location is exceedingly rare, with less than ten cases described[ 81 , 82 ]. Oesophageal Paget’s disease (OPD) always arises in the setting of Barrett’s oesophagus[ 81 , 82 ].…”

“…Clinical features and diagnostic approach: Typically, patients complain of progressive dysphagia, eventually accompanied by chest discomfort similar to reflux oesophagitis[ 81 , 82 ]. EGDS is a first-line diagnostic tool.…”

“…Pathological features: The microscopic features of OPD include the presence of intraepithelial nests and isolated cells with large round-shaped cytoplasm spreading within the entire squamous epithelial layer. These cells usually invade the mucosal and submucosal ducts multifocally, indicating adenocarcinomatous differentiation[ 82 ]. One of the main differential diagnoses is primitive oesophageal melanoma: Positive immunohistochemistry staining for mucins, cytokeratin 18 and E-cadherin, together with negative staining for S100, will help distinguish OPD from oesophageal melanoma[ 82 ].…”