Retroperitoneal Cystic Lymphangioma: A Diagnostic and Surgical Challenge

Abstract

A lymphangioma is a benign proliferation of lymph vessels, producing fluid-filled cysts that result from a blockage of the lymphatic system. The incidence of abdominal lymphangiomas is unknown; however they account for from 3% to 9.2% of all pediatric lymphangiomas, with retroperitoneal lymphangioma representing less than 1% of abdominal lymphangiomas. Due to rarity, preoperative diagnosis is often difficult.

Lymphangioma of the small bowel mesentery in a 7-year-old girl: a report of a case investigated with ultrasonography, elastosonography and computed tomography

“…Lymphangioma is a mass-forming lesion which usually presents in the first few years of life with 50-60 % manifesting by age 1 year and 90 % by age 2 years, while in our case the little patient was 7-year-old [4,6,7]. Although the incidence of abdominal LAs is unknown, they represents for 3-9.2 % of all pediatric LAs, and among these, LA of the small bowel mesentery, has been described in less than 1 % of all LAs [3,6,7].…”

“…Histologically, LAs can be distinguished into three types: capillary or simple, cavernous, and cystic. Cystic spaces are seen for both cystic and cavernous LA and may contain chylous, serous, hemorrhagic, or mixed fluid [3,4]. In this case study, sectioned mass presented multicystic aspect with cysts containing milky white fluid supporting LA, in which the white color is due to the great presence of lymphoid cells.…”

“…In this case study, sectioned mass presented multicystic aspect with cysts containing milky white fluid supporting LA, in which the white color is due to the great presence of lymphoid cells. Microscopically, dilated lymphatic spaces of varying size were associated with smooth muscle cells and lymphoid aggregates clearly characteristic of cystic LA [4,7]. Intraabdominal LA usually presents with abdominal distension, a palpable abdominal mass, or acute intestinal obstruction when LA induces volvulus of the small bowel [3,4,7].…”

“…Lymphangioma (LA) is a benign proliferation of thinwalled lymphatic tissue believed to originate from the early failure of communication of lymphatic spaces with the normal main draining lymphatic vessels, thus being considered a congenital malformation rather than an acquired tumor [4,6,7]. It is usually found in the head and neck (50-75 %) and axillary (20 %) regions during the first few years of life, while other locations are more rare [4,7].…”

“…It is usually found in the head and neck (50-75 %) and axillary (20 %) regions during the first few years of life, while other locations are more rare [4,7]. Among these ones, LA of small bowel mesentery represents less than 1 % of all cases described in literature [7].…”