Baylor University Medical Center, Baylor Scott & White Health Dallas, TX
Introduction: Epidermoid metaplasia (EM) of the esophagus (esophageal leukoplakia) is a rare lesion that usually affects middle-aged to elderly women, and is strongly associated with esophageal squamous cell carcinoma (ESCC). EM is defined histologically by hyperorthokeratosis, a marked thickening of the superficial layer of anuclear esophageal squamous cells, along with a prominent granular layer. Endoscopically, EM appears as white esophageal plaques that cannot be rubbed off. We report a case of a 59-year-old man with asymptomatic EM and ESCC found during assessment for iron deficiency anemia.
Methods: A 59-year-old African-American male smoker was found to have iron deficiency anemia. He had endoscopy at an outside hospital, which showed an arteriovenous malformation likely to be the cause of his iron deficiency anemia. Endoscopy also revealed unusual esophageal lesions with biopsies showing epidermoid metaplasia, and he was referred to us for further evaluation. Our esophagoscopy demonstrated long, whitish, nearly hemi-circumferential plaques of thickened mucosa with a cobblestone appearance from 26 to 34 cm from the incisors, and a smaller, patchy area from 34 to 36 cm (Image 1). Narrow-band imaging (NBI) revealed a cerebriform mucosal pattern with a decreased vascular pattern (Image 2). NBI also showed similar changes, not apparent by white light endoscopy, extending from 21 to 26 cm from the incisors. Biopsies confirmed epidermoid metaplasia (Image 3). Due to the worrisome appearance and known association of EM with ESCC, we performed a step-wise endoscopic submucosal dissection, which demonstrated areas of low-grade dysplasia and areas of ESCC. Discussion: Oral leukoplakia is common, whereas EM is rare. One study found only 2 cases of EM after reviewing esophageal biopsies from 1,048 patients, and identified no new cases during a 6-month, prospective multicenter study. A rare subset of oral leukoplakia exhibits orthokeratotic dysplasia, a histologic pattern identical to that of esophageal EM and, like EM, this form of oral leukoplakia is associated with SCC. In patients with esophageal EM, high-grade dysplasia or cancer within or surrounding EM lesions has been found in up to 17% of cases. Typically, EM is found in women with dysphagia and risk factors of smoking and alcohol use. Our patient was not typical in that he was an asymptomatic man, but this case highlights the strong association of esophageal EM with ESCC.
Esophagus with a cobblestone mucosa appearance.
NBI with near focus showing a cerebriform mucosal pattern with decreased vasculature.
Esophageal squamous mucosa with thickened keratin layer (hyperorthokeratosis) and a prominent thickened granular layer with basophilic keratohyalin granules (A&B). Esophageal squamous epithelium with loss of nuclear polarity, nuclear atypia (including hyperchromatic change, high N/C ratio, and pleomorphism) involving the lower one-third of the epithelium without crossing the basement membrane into the lamina propria (C&D). Low (black arrow) and high power views showing nests of atypical squamous cells with surrounding desmoplastic response in the lamina propria of the esophageal squamous mucosa (E&F). Hematoxylin/eosin stain ×100 (A), x400 (B), x20 (C), x100 (D), x40 (E), and ×100 (F).
Disclosures: Ashton Ellison indicated no relevant financial relationships. Eitan Podgaetz indicated no relevant financial relationships. Nitin Kondapalli indicated no relevant financial relationships. Atin Agarwal indicated no relevant financial relationships. Anh Nguyen indicated no relevant financial relationships. Vani Konda indicated no relevant financial relationships.