Esophageal Paget\u2019s Disease Secondary to Hypopharyngeal Carcinoma: a Case Report

Abstract

Paget’s disease is defined as neoplastic cells of glandular differentiation infiltrating squamous epithelium [1]. In essence, it represents a population of malignant mucin-producing cells which sit in amongst benign squamous cells within skin or mucosa. These cells can arise secondary to a primary carcinoma, which may have so called ‘Pagetoid spread’ of malignant cells at the periphery of the lesion, or more rarely, Paget’s cells can arise de novo within the squamous epithelium, the pathogenesis of which is not yet understood. Having been first described in the breast by Sir James Paget in 1874, extramammary Paget’s disease is an entity most commonly seen in the vulva and anus but it is known to occur rarely in the esophagus, almost exclusively in the context of invasive esophageal adenocarcinoma. Paget’s cells within esophageal squamous epithelium are found in 4.9% of invasive adenocarcinoma cases and are most commonly associated with diffuse, poorly differentiated adenocarcinoma with dyscohesive malignant cells [2–5]. Few case reports are present in the literature to describe Paget’s cells within the esophagus but those that exist include cases involving mucous gland carcinoma, adenosquamous carcinoma and adenocarcinoma arising on a background of Barrett’s esophagus [2–5]. We present a case in which widespread esophageal Paget’s disease arises secondary to poorly differentiated carcinoma of the hypopharynx. A literature search did not yield any similar cases.