Esophageal Stenosis Caused by a Rare Entity

Abstract

Question: A 45-year-old man presented in our surgical department with progressive dysphagia for solids and liquids and weight loss over the last 6 month, he had a long history of tobacco abuse (25 pack-years), but no history of trauma or alcohol use. Physical examination showed his cachexia, but was otherwise unremarkable without palpable lymphadenopathy. He had been treated with long-term bouginage over 4 years for a long segment esophageal stenosis starting 30 cm distal to the dental arch. Repeatedly performed biopsies over that time course had been nondiagnostic, with only chronic inflammation and hyperkeratosis. He presented in our institution owing to shorter bouginage intervals and weight loss. Upper endoscopy showed a thickened, white, contact-vulnerable, exophytic, and lumen-constricting polypoid mass in the esophagus over a distance of 15 cm (Figure A; star = esophageal lumen). Multiple deep biopsies were taken; they revealed keratosis and chronic inflammation but no evidence of malignancy. The subsequent computed tomography with contrast enhancement revealed a long-segment thickening of the thoracic esophagus (Figure B, arrows) without any lymphadenopathy or metastases. What is the most likely diagnosis? Look on page 871 for the answer and see the Gastroenterology web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The verrucous carcinoma (VC) of the esophagus is a rare, well-differentiated, squamous cell carcinoma with about 20 cases described worldwide. It is associated with long-term local inflammation; tobacco abuse1Alkan A. Bulut E. Gunhan O. et al.Oral verrucous carcinoma: a study of 12 cases.Eur J Dentistry. 2010; 4: 202-207Crossref PubMed Google Scholar and recent studies suggest a potential role for human papillomavirus in carcinogenesis.2Viera C.L. Lopes J.C. Velosa J. A case of esophageal squamous cell carcinoma with positive HPV 11.Gastroenterol Hepatol. 2013; 36: 311-315Crossref PubMed Scopus (12) Google Scholar Despite its high degree of differentiation and slow growth, the outcome of patients with VC is unsatisfactory owing to late diagnosis and perioperative complications.3Osborn N.K. Keate R.F. Trastek V.F. et al.Verrucous carcinoma of the esophagus: clinicopathophysiologic features of a rare entity.Dig Dis Sci. 2003; 48: 465-474Crossref PubMed Scopus (42) Google Scholar Local invasion and its spread within the submucosa and paraesophageal layer leave it invisible for superficial biopsies, which usually show nonspecific acanthosis and hyperkeratosis associated with acute or chronic inflammation. The tumor invades the surrounding tissue, grows locally destructive, and has a very low potential for metastasis. Typical endoscopic appearances—mostly the only “proof” for this malignancy—include wart-like polypous, exophytic, spiked, or cauliflower like masses.3Osborn N.K. Keate R.F. Trastek V.F. et al.Verrucous carcinoma of the esophagus: clinicopathophysiologic features of a rare entity.Dig Dis Sci. 2003; 48: 465-474Crossref PubMed Scopus (42) Google Scholar Surgery is adequate therapy for early stages that are locally resectable; patients with esophageal VC do not benefit from chemotherapy. Our patient underwent thoracoabdominal esophagectomy. Intraoperatively, we found a thin, dilated, proximal esophagus with a thick wall in the stenotic area and inflamed adhesion to the mediastinum. The macroscopic pathological specimen showed the strong hyperkeratotic, exophytic growth of the tumor (Figure C, arrows); final histology revealed the VC of the esophagus pT2, pN0, L0, V0, Pn0, R0, G1. The microscopic image displays the invasive component of the squamous cell carcinoma (Figure D, arrow). The patient was dismissed after an uneventful postoperative course on postoperative day 15. VC of the esophagus is not only a rare entity, its preoperative diagnosis also is extraordinary challenging and relies on clinical suspicion induced by typical endoscopic appearance. An esophageal resection should be performed in case of severe, progressive dysphagia in combination with typical endoscopic appearance even without histologic proof, because definitive diagnosis can mostly first be seen in the resected specimen.