Endoscopic submucosal dissection of esophageal lymphangioma: a case report with a review of the literature

Abstract

Esophageal lymphangioma is a very rare disease. We report a case of esophageal lymphangioma successfully treated with endoscopic submucosal dissection (ESD), which yielded definitive histological diagnosis and symptom relief. ESD offers a better option for definitive diagnosis as well as complete resection of large esophageal lymphangiomas with flat configuration.

Oesophageal lymphangioma: an exceedingly rare tumour

“…Although a multicystic pattern within the third layer or submucosa is considered a feature of lymphangioma,4 not all lymphangiomas have this internal echo structure,4 23 because the tumour structure may vary in accordance with the size of the dilated lymphatic vessels 4. This is most likely the reason for the absence of the typical endosonographic pattern in our case.…”

“…The clinical presentation of this entity varies, depending on the location and size of the tumour 23. The vast majority of patients are asymptomatic, and the tumour is incidentally discovered during endoscopic investigation for symptoms not related to it,8 as in the current case.…”

“…On endoscopic examination, an oesophageal lymphangioma appears as a pale, pink, white or yellowish translucent subepithelial polypoid mass projecting into the lumen, and covered with normal mucosa; it is compressible when probed with biopsy forceps 4 18 23…”

“…EUS is the most useful examination for distinguishing lymphangioma from leiomyoma and other subepithelial tumours because it accurately shows the cystic nature of lymphangioma and whether the oesophageal muscles are intact, which is necessary for the tumour to be amenable to endoscopic resection 4 15 18 23. Although a multicystic pattern within the third layer or submucosa is considered a feature of lymphangioma,4 not all lymphangiomas have this internal echo structure,4 23 because the tumour structure may vary in accordance with the size of the dilated lymphatic vessels 4.…”

“…Because malignant transformation of oesophageal lymphangioma has never been reported in the literature, the management of this tumour can be conservative if the patient is asymptomatic and if a histological diagnosis has already been established 18 23…”