Clinical Challenges and Images in GI

Abstract

Question: A 65-year-old man was admitted because of progressive dysphagia, resulting in a 25-kg weight loss over the last 5 months. Five years before, he had an episode of sore throat, which subsided after 2 months. He had undergone partial gastrectomy for a benign gastric ulcer and was been found to have dilated cardiomyopathy, which prompted the implantation of a biventricular cardiac pacemaker. Physical examination and routine laboratory tests were within normal limits. A barium swallow study demonstrated a filling defect from the cervical esophagus down to the stomach, with a corrugated appearance (Figure A). The esophagogastroscopy showed a mass protruding into the esophagus (Figure B). Endoscopic biopsy done at the proximal site of the lesion showed a normal esophageal mucosa. Computed tomography (CT) is shown in Figure C. What might be the diagnosis and the appropriate therapeutic approach?View Large Image Figure ViewerDownload Hi-res image Download (PPT)View Large Image Figure ViewerDownload Hi-res image Download (PPT) Look on page 1019 for the answer and see the Gastroenterology website (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. The CT scan with hydrosoluble oral contrast showed a polypoid lesion of 5 cm in diameter and 23 cm long arising in the cervical esophagus, occupying the entire esophagus and prolapsing through the esophagogastric junction into the stomach (Figure D). Because of size of the mass and the presence of a short, hypervascularized stalk, an endoscopic approach was thought to be inappropriate. In consideration of the patient's clinical conditions, esophagectomy was ruled out. It was decided to use a left side cervical approach, through which the pedicle was resected; however, it was not possible to take the mass out. Thus, a right thoracotomy was performed, followed by 5-cm longitudinal incision of the esophageal wall to enter the lumen from which the mass was taken out (Figure E). Histologic examination was consistent with liposarcoma. The patient had an uneventful postoperative course without any complication and was discharged on postoperative day 12.View Large Image Figure ViewerDownload Hi-res image Download (PPT) At 6 months follow-up, a CT scan of the chest and upper abdomen as well as an upper GI endoscopy showed no tumor recurrence or distal metastases. Esophageal liposarcoma is an extremely rare entity, the present case representing the 21st described in the literature to date.1Will U. Lorenz P. Urban H. et al.Curative endoscopic resection of a huge pedunculated esophageal liposarcoma.Endoscopy. 2007; 39: E15-E16Crossref PubMed Scopus (16) Google Scholar, 2Garcia M. Buitrago E. Bejarano P.A. et al.Large esophageal liposarcoma: a case report and review of the literature.Arch Pathol Lab Med. 2004; 128: 922-925PubMed Google Scholar, 3Mandell D.L. Brandwein M.S. Woo P. et al.Upper aerodigestive tract liposarcoma: report on four cases and literature review.Laryngoscope. 1999; 109: 1245-1252Crossref PubMed Scopus (37) Google Scholar, 4Boggi U. Viacava P. Naccarato A.G. et al.Giant pedunculated liposarcomas of the esophagus: literature review and case report.Hepatogastroenterology. 1997; 44: 398-407PubMed Google Scholar Neither barium swallow nor esophagogastroscopy are helpful diagnostic tools. Only CT scan with hydrosoluble contrast swallow allowed us to define the anatomic relationship of the mass with the esophageal wall. Almost all cases in the literature have been approached by a cervicotomy, but in 15 cases a second access such as a laparotomy or thoracolaparotomy was necessary to remove the tumor, as in our case. Only 4 patients underwent esophagectomy. Unfortunately, avoiding esophagectomy increases the risk of an R1 resection. Esophageal liposarcoma management remains vague; therapeutic options range from less invasive endoscopic excision to radical esophagectomy.