Kaiser Permanente Los Angeles Medical Center Los Angeles, CA
Introduction: Epithelioid angiosarcoma is a rare and aggressive soft-tissue endothelial cell neoplasm that accounts for less than 1% of sarcomas and rarely occurs in the gastrointestinal tract. We report a case of a 41-year-old female without prior medical history who presented with progressive dysphagia and unintentional weight loss.
Case Description/Methods: Initial upper endoscopy found to have a narrowed gastroesophageal junction (GEJ) that required balloon dilation to 10mm and the use of a pediatric upper endoscope to traverse. Only thickened gastric folds at the cardia were seen, which were biopsied. Histology revealed chronic active moderate gastritis with Helicobacter pylori, and treated with triple therapy. However, due to ongoing dysphagia a contrast enhanced computed tomography (CT) of the chest was obtained and demonstrated thickening of the distal esophageal wall. Next, an endoscopic ultrasound (EUS) was performed and demonstrated unusual thickening of the distal esophageal wall. Fine needle biopsy (FNB) revealed high-grade esophageal epithelioid angiosarcoma of the distal esophagus. She was treated with neoadjuvant chemo- and radiotherapy followed by a robotic laparoscopic Ivor Lewis esophagectomy. The patient has been disease free after one month of follow up.
Discussion: This case highlights the importance of timely recognition and diagnosis of dysphagia caused by pseudo-obstruction. Timely CT and EUS/FNB should be performed to evaluate for less common entities.
