Verrucous carcinoma of the esophagus. A case report

Verrucous carcinoma (VC) of the esophagus is a rare variant of squamous cell carcinoma and only 20 cases have so far been reported in the international literature. The neoplasm is usually highly differentiated, presents aslow growth pattern with invasion of surrounding organs rather than blood-borne metastases. Recently, a causative role of human papillomavirus (HPV) has been hypothesized. The case of a patient affected with locally advanced VC of the esophagus and treated by means of local antiviral therapy is reported. A 41-year-old male patient was admitted to our institution for persistent atypical thoracic pain. The imaging techniques (thoracic and abdominal CT scans; upper GI endoscopy; hydrosoluble contrast swallow; endoscopic US) revealed a cauliflower-like protruding esophageal mass, active mucosal mycosis, multiple ulcerations of the distal esophagus, as well as 2 broncho-esophageal fistulas. The neoplasm extended beyond the esophageal wall, infiltrating surrounding cervical and mediastinal organs and the patient presented with secondary esophageal achalasia and right bilobar pneumonia. The histologic specimen was consistent with VC of the esophagus and the presence of HPV infection was detected by means of qualitative PCR assay. The patient was deemed not fit for surgery and a local antiviral treatment with hydroxy-phosphonylmethoxypropyl-cytosine 5 mg/kg a week was started. After initial response to treatment, the patient presented with sudden progression leading to further broncho-esophageal fistula treated with endoscopic stent placement and ultimate death 6 months after referral to our center. In keeping with international data, our case confirms that esophageal VC has a highly unfavorable outcome, despite its high degree of differentiation and slow growth pattern. The long natural history, the lack of specific symptoms and the presence of coexisting esophageal diseases delay the diagnosis and account for the local advancement of this malignancy. Surgery is the option of choice for early stage lesions and advanced VC does not seem to benefit from current chemotherapeutic regimens. The causative role of HPV and the advancements of molecular pharmacology might allow for effective treatment in high-risk patients.