Lymphangioma of the small bowel mesentery: A case report and review of the literature

Abstract

Lymphangioma is a rare benign condition characterized by proliferation of lymphatic spaces. It is usually found in the head and neck of affected children. Lymphangioma of the small-bowel mesentery is rare, having been reported for less than 1% of all lymphangiomas. Importantly, it can cause fatal complications such as volvulus or involvement of the main branch of the mesenteric arteries, requiring emergency surgery. Moreover, the gross and histopathologic findings may resemble benign multicystic mesothelioma and lymphangiomyoma. Immunohistochemical study for factor VIII-related antigen, D2-40, calretinin and human melanoma black-45 (HMB-45) are essential for diagnosis. Factor VIII-related antigen and D2-40 are positive in lymphangioma but negative in benign multicystic mesothelioma. HMB-45 shows positive study in the smooth-muscle cells around the lymphatic spaces of the lymphangiomyoma. We report a case of small-bowel volvulus induced by mesenteric lymphangioma in a 2-year-and-9-mo-old boy who presented with rapid abdominal distension and vomiting. The abdominal computed tomography scan showed a multiseptated mass at the right lower quadrant with a whirl-like small-bowel dilatation, suggestive of a mesenteric cyst with midgut volvulus. The intraoperative findings revealed a huge, lobulated, yellowish pink, cystic mass measuring 20 cm × 20 cm × 10 cm, that was originated from the small bowel mesentery with small-bowel volvulus and small-bowel dilatation. Cut surface of the mass revealed multicystic spaces containing a milky white fluid. The patient underwent tumor removal with small-bowel resection and end-to-end anastomosis. Microscopic examination revealed that the cystic walls were lined with flat endothelial cells and comprised of smooth muscle in the walls. The flat endothelial cells were positive for factor VIII-related antigen and D2-40 but negative for calretinin. HMB-45 showed negative study in the smooth-muscle cells around the lymphatic spaces. Thus, the diagnosis was lymphangioma of the small bowel mesentery with associated small bowel volvulus.

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].…”

“…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.…”

“…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].…”

“…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]. The clinical presentation of abdominal LAs is polymorphic, varying from the incidental discovery of an abdominal cyst, which becomes symptomatic when reaches large size, up to the signs and symptoms of acute abdomen, with abdominal pain and bowel occlusion [3].…”