Oesophageal lymphangioma: an exceedingly rare tumour

Abstract

An asymptomatic 57-year-old man was referred for evaluation of a 10 mm pale pink subepithelial polypoid lesion of the distal oesophagus; the lesion was found incidentally during an upper gastrointestinal endoscopy. The endoscopic ultrasound (EUS) revealed a heterogeneous, although primarily hypoechogenic, well-circumscribed lesion, located in the submucosa. Owing to the absence of endosonographic features, which would have allowed for a precise diagnosis, EUS-guided fine-needle aspiration was performed; however, the cytology was inconclusive because of the lack of cellular representation. We chose to remove it by cap-assisted endoscopic mucosal resection. However, due to its shape and deep location, complete removal was not achieved. A macrobiopsy was taken, and histological examination, of all oesophageal layers except the adventitia, revealed many irregularly dilated lymphatic vessels, beneath normal squamous epithelium. These findings are consistent with the diagnosis of lymphangioma. A follow-up EUS did not reveal any changes. The patient is currently asymptomatic and is under surveillance.

Histopathological landscape of rare oesophageal neoplasms

“…Background: Lymphangiomas (LAs) are benign soft tissue tumours that should actually be considered congenital malformations rather than neoplasms[ 30 ]. An oesophageal location of LAs is exceedingly rare, with only 22 cases reported worldwide[ 31 ]. Notably, even though the diagnosis of LAs is typically made in children younger than two years of age, all oesophageal cases have been described in the distal oesophagus in middle-aged men[ 31 ].…”

“…An oesophageal location of LAs is exceedingly rare, with only 22 cases reported worldwide[ 31 ]. Notably, even though the diagnosis of LAs is typically made in children younger than two years of age, all oesophageal cases have been described in the distal oesophagus in middle-aged men[ 31 ].…”

“…The adequate diagnostic assessment requires the use of EUS examination of the oesophageal wall, which shows the cystic nature of LAs. When EUS findings are not typical, the definitive diagnosis of oesophageal LAs requires histological examination[ 31 ].…”

“…Pathological features: Oesophageal LAs are characterised by thin walled, dilated lymphatic channels lined by flattened endothelium, which can be empty or filled with chylous or serous fluid, with the occasional presence of lymphocytes or erythrocytes. Additionally, these tumours are frequently surrounded by lymphocytic aggregates[ 31 - 33 ].…”

“…Treatment: Small LAs ( i.e ., less than 2.5 cm in size) that leave the oesophageal muscles intact can be managed by ESD. Surgical treatment is needed when tumours are large and involve the muscular layer[ 31 ].…”