Verrucous Squamous Cell Carcinoma of the Esophagus Presenting as a Tracheo-esophageal (T-E) Fistula

Abstract

Purpose: A 53-year-old African-American woman with a history of hypertension, asthma, and gastroesophageal reflux disease (GERD) was admitted for 3 days of progressive dyspnea. She first noted GERD symptoms 7 years prior. An esophagogastroduodenoscopy (EGD) at that time revealed an esophageal mass that was biopsied and found to be benign. For the last 2 months, she noted worsening heartburn and regurgitation associated with productive cough and wheezing. She denied fever, chills, or sick contacts. Pleuritic chest pain and severe dyspnea prompted admission. On exam she was a thin tachypneic woman. She was afebrile, saturating 97% on room air. She had clubbing, supraclavicular retractions, bilateral wheezing, and rales. Lab tests revealed normal hemoglobin (12 g/dL) and an elevated white count (15,000) with 91% neutrophils. Computed tomography (CT) showed bilateral lower lobe consolidation, ground glass opacities, and nodular mural thickening of the mid-distal esophagus distended with debris. The impression was aspiration pneumonia with possible achalasia/pseudoachalasia. The patient was treated with antibiotics and an endoscopy was planned. On EGD, a necrotic mass covered with slough was seen in the esophagus extending 30-36 cm from the incisors. There was a diverticulum in the distal esophagus close to the Z-line. Biopsies revealed hyperkeratosis with no features of malignancy. She was placed on esomeprazole 40 mg twice a day and discharged with plans for follow-up with endoscopic ultrasound when her respiratory status improved. One month later she was readmitted with aspiration pneumonia and acute dyspnea. EGD for gastrostomy placement was done after strict NPO. At this time T-E fistula was suggested. A coronal re-evaluation of the CT scan confirmed the mural thickening of the mid-distal esophagus connected to the tracheal carina. Repeat EGD for stent placement showed a large fistula 35 cm from the incisors and distal to it a stricture. A 23 mm to 15 cm-long covered metal stent was placed. Despite excellent positioning, the patient continued to aspirate. She was transferred to a tertiary institution. Bronchoscopy also revealed an erosive mass. A fistula of the left bronchus was noted, requiring two endobronchial stents. Tracheal biopsy of the mass was negative for malignancy. Due to the high suspicion, the patient underwent open lung biopsy. This revealed an epithelial verrucous squamous cell carcinoma. It is a rare well-differentiated squamous carcinoma that divides slowly, invades locally, and metastasizes rarely. Diagnosis requires deep biopsies and a high index of suspicion.