[Cystic lymphangioma of the ileum]

Cystic lymphangioma is a rare benign neoplasm of lymphatic origin of which literature data are limited. It is mainly described as a disease of children and is considered to be a congenital malformation. Primary localisation is in the cervical and axillary regions; however, there may also be abdominal manifestations, in which case this entity is rarely contemplated in the differential diagnosis due to its diverse clinical manifestations. Hereby we present the case report of a 10-year-old girl who was admitted to the hospital due to cramping abdominal pain, behind which the abdominal ultrasound examination revealed a tumour involving the ileum and the ileal mesentery, consisting of numerous cystic masses. The lesion led to a volvulus and resection of the affected bowel segment was performed. Macroscopic examination revealed a cystic tumour with brownish and cluttered content. Microscopically, cystically dilated lymphatic vessels were visible in the lamina propria, tunica submucosa, tunica muscularis, and surrounding mesenteric adipose tissue, lined by atypia-free, flat endothelial cells. Immunohistochemistry revealed diffuse, strong CD31, as well as CD34 positivity. The case was concluded as cystic lymphangioma. The patient has been asymptomatic since surgery. In this article, we present a very rare abdominal manifestation of cystic lymphangioma, with literature review, for the first time in Hungarian literature. The diverse clinical presentation reflected in the international studies shows that the recognition and the differential diagnosis of this lesion may be difficult, but definitive diagnosis can be set by imaging techniques and histological, and if necessary, immunohistochemical studies. Laparoscopic resection is used as an international gold standard, after which good prognosis is expected. Orv Hetil. 2024; 165(29): 1135–1139.