Lymphangioma of Esophagus Presenting with Dysphagia: A Case Report

Abstract

Purpose: Lymphangioma of the esophagus is a benign vascular tumor that rarely occurs in the upper GI tract. We report a rare case of lymphangioma of the esophagus and our experience with endoscopic resection of this lesion. A 61 year old Hispanic man with history of dysphagia was referred for evaluation of a polypoid lesion in the mid-esophagus. An EGD done by his gastroenterologist a year earlier demonstrated a submucosal polypoid lesion in the mid esophagus. The patient was placed on a PPI and was referred for an EUS. EGD showed a 1 cm submucosal lesion at 25 cm from incisors. It was round, with normal appearing overlying mucosa and was easily compressed with cold forceps. EUS demonstrated a 13 × 5 mm anechoic lesion arising from the second echo layer corresponding to submucosal space. Next, using cold standard forceps, multiple biopsies were taken in a “bite and bite” tunneling method with obliteration of the lesion. Histology showed cavernous lymphangioma of the esophagus. His dysphagia improved after removal of the lesion. Discussion: Lymphangiomas are benign lesions that arise from the cystic dilatation of hamartomas in deep lymphatic tissue and are rarely found in the GI tract (1%). The esophagus is the most unusual location for this lesion. The diagnosis of lymphagioma is usually made on histopathology. The masses are composed of enlarged channels lined by lymphatic endothelial cells surrounded by a loose myxoid stroma. On gross examination, they are usually pale and smooth, with a multicystic cut surface and a clear yellowish fluid exudate. The common tools to establish the diagnosis are esophagram and EGD. Lymphangiomas appear as a translucent, yellowish, easily compressible mass lesions that are < 5 mm. Standard biopsies are often normal as the lesions are submucosal. EUS may the most useful tool for distinguishing a lymphangioma from other submucosal tumors because it aids in differentiating submucosal lesions from lesions that arise from the muscular layers of the esophagus. The treatment of esophageal lymphangioma is conservative in asymptomatic patients. For symptomatic pateints, response to surgical or endoscopic removal of these tumors is excellent. However, a large tumor with symptoms or a lesion suspicious for malignancy requires surgical resection. Determining the extent of the tumor through the esophageal wall may be difficult during resection as the lymphangioma is soft and compressible. Conclusion: Lymphangioma of the esophagus is a rare submucosal tumor, generally small and benign in nature. Endoscopic ultrasound is an excellent tool for diagnosis of these lesions. Our experience reaffirms that endoscopic excision of such lesions is safe and appears to be without major complications.