Esophageal Verrucous Carcinoma: A Case Report and Review of a Rare Disease

Abstract

Purpose: A 64-year-old male presented with progressive dysphagia to solids and liquids and associated unintentional weight loss. EGD revealed an exophytic, circumferential mass in the distal esophagus extending through the GE junction involving the gastric cardia (Figure 1A). Biopsies from the mass showed a squamous papilloma with parakeratosis and reactive cytological atypia. EUS demonstrated the mass to be transmural, invading through the serosa and abutting the left pleura (Figure 1B). Suspicious lymphadenopathy was noted in the subcarinal, paraesophageal, and celiac regions. Cytology from FNA of subcarinal nodes returned with insufficient tissue. The mass was intensely FDG-avid on PET-CT scan. Because of significant concerns for malignancy despite biopsy results, the patient underwent a transhiatal esophagectomy, mediastinal and abdominal lymphadenectomy, and creation of a cervical esophagogastrostomy. Operative pathology returned consistent with invasive, well-differentiated squamous cell carcinoma involving the distal esophagus and proximal stomach invading through the muscularis propria into the adventitia. All 34 lymph nodes sampled were negative for metastatic disease. Surgical margins were negative. Final staging was T3N0. The patient has had no evidence of disease recurrence in follow-up over the past year. Esophageal verrucous carcinoma (EVC) is an extremely rare variant of squamous cell carcinoma with few cases reported in the literature since initially being described in 1967. As in our case, a history of tobacco and alcohol use is often obtained. The association between EVC and human papilloma virus is not firmly established. Most commonly, EVC has a wart-like, exophytic appearance with white coloration. Concomitant Candida esophagitis is frequently seen. EVC presents a diagnostic dilemma as routine mucosal biopsies are typically negative for carcinoma despite the endoscopic appearance of malignancy. EVC tends to spread superficially and grow slowly with low rates of metastasis even in patients with prolonged symptomatology. Care must be taken to avoid over-staging by EUS, which can occur due to associated adjacent inflammatory changes, so as to not preclude curative surgical resection as the treatment of choice for EVC.Figure