Pseudoachalasia due to an Unusual Esophageal Tumor

Abstract

There have been cases of patients who are diagnosed with achalasia when they actually have pseudoachalasia. Many times the pseudoachalasia is due to an esophageal squamous cell carcinoma or adenocarcinoma. This is a case of pseudoachalasia with an unusual tumor finding. Case Report: A 51 year old male with a history of achalasia presented with fatigue, melena, intermittent fevers, and weight loss for the past 3 weeks. Labs were notable for a Hgb of 5.7, WBCs 11.1, and albumin 1.8. He had an esophageal manometry a year prior at another hospital, read as a gastroesophageal outflow obstruction likely representing achalasia. There, he also underwent an EGD showing mild esophagitis. Barium esophagram on admission to Tampa General showed likely stricture of the proximal and distal esophagus (less likely mass) with dilation of rest of the esophagus. EGD revealed a proximal pouch in the upper esophagus. The esophageal mucosa appeared normal, but was not distending well with air. A 5-6 cm hard, necrotic mass was found in the cardia, which appeared to be on a stalk originating from the gastroesophageal junction. Biopsy of the mass showed fragments of ischemic fibrinous necrotic debris with scattered inflammation, but no malignant cells. CT chest with contrast showed a large intraluminal esophageal mass, measuring 20.2 cm, extending from the level of the clavicles to the gastroesophageal junction. EUS revealed a longitudinal mass from 20-40 cm which originated from the muscularis mucosa (layer 2). FNA showed scattered squamous cells. The decision was made to proceed with surgery. The tumor was completely excised from the submucosal layer without an esophagectomy. The final pathology revealed well-differentiated liposarcoma with myxoid change/atypical lipomatous tumor which formed a 16.9 cm mass- stage pT2b pNX. The patient did well post operatively, without chemo or radiation, and returned to a regular diet with no dysphagia. This case highlights the importance of continuing work up after a diagnosis of possible achalasia when there are still uncertainties. It is unclear if the tumor was missed on the original EGD or obvious signs were not present yet. A review of the literature found no cases of pseudoachalasia due to esophageal liposarcoma. Large esophageal liposarcomas are exceedingly rare, often resulting in esophagectomies. It is unique that this tumor could be fully resected from the submucosal layer, avoiding an esophagectomy.Figure: Esophageal liposarcoma after surgical resection.Figure: CT chest with arrow pointing to esophageal liposarcoma.Figure: Liposarcoma extending from the gastroesophageal junction on EGD.