Paget's disease of the oesophagus associated with mucous gland carcinoma of the lower oesophagus

Aim: To report a rare case of oesophageal Paget's disease and its rarer combination with submucosal gland carcinoma of the lower oesophagus. Methods and results: A 74-year-old Saudi female was admitted with the complaint of dysphagia. Endoscopic examination showed an ulcerated tumour at the gastro-oesophageal junction. Initial biopsy showed an undifferentiated carcinoma with pagetoid spread in the oesophageal stratified squamous epithelium. Oesophago-gastrectomy specimen showed a lobulated, poorly differentiated mucous gland carcinoma at the gastro-oesophageal junction. The tumour showed focal acinar differentiation and obvious cancerization of the submucosal glands, somewhat similar to the breast lobular carcinoma in situ. One of the isolated and cancerized submucosal glands also showed carcinoma in situ of its duct. Oesophageal surface epithelium showed extensive pagetoid spread, both over and away from the main tumour. The pagetoid tumour cells showed selective positivity for cytokeratin 7, cytokeratin Cam 5.2, BerEP4 and to a lesser extent for CEA. Conclusions: As far as we know, this is the fifth case report of oesophageal Paget's disease and the first report of its association with the underlying mucous gland carcinoma.