Primary intestinal lymphangiomatosis of the ileum in an adult-the role of surgical approach

Abstract

Lymphangioma is a rare benign tumor that occurs due to abnormalities occurring during lymphatic development. It is usually seen in children and young adults. The incidence of lymphangiomas in the gastrointestinal tract is very low. Here we describe the case of 43-year-old woman with lymphangioma of the ileum with infiltrative polyposis-like appearing lesions diagnosed by capsule endoscopy and treated with segmental resection of affected intestinal part with laparotomy. Lesions involving mesentery and ileum were confirmed by pathology. After surgery, the patient's symptoms improved. No further therapy was needed because of the benign manner of the lesions. Patient had no symptoms in 10 months follow-up after surgery.

Figure 1:

An abdominal gray-scale ultrasound image obtained with a linear transducer clearly depicts multiple anechoic cystic nodularities (arrowheads) in the intestinal wall protruding the fluid-filled lumen (*).

Figure 2:

An axial CT image obtained with intravenous and oral contrast material shows abnormal wall thickening of ileal loops (arrows). Please note that the multiple nodular formations of ileal wall protruding the contrast-filled intestinal lumen (*). Abdominal CT scan also demonstrates a mass-like diffuse expansion of mesenteric root with low attenuation (arrows).

Figure 3:

(A) Expanded cystic vascular lesion focally extending to the intestinal mucosa is seen (H&E, 4× magnification). (B) High power view of the lesion (H&E, 20× magnification).

Figure 4:

Immunohistochemically the endothelial cells, lining the vascular structures, were reactive with D2-40 (A), CD31 (B) and ERG (C).