Cystic retroperitoneal lymphangiomas in adults

Lymphangioma (LA) is a rare benign tumour of the lymphatic tissue, most common in the neck and head, and clinically manifests itself mostly in childhood. Within this group, intra-abdominal and retroperitonal LA are the rarest tumours, especially when occurring in adults. We report four LAs localized in the retroperitoneum of patients aged between 28 and 72 years. One of these tumours infiltrated the transverse mesocolon and greater omentum, others were situated in the left retroperitoneum and retroperitoneally at the duodeno-jejunal flexure, and in the retrosplenal and retropancreatic area. Diagnosis was made by light microscopy supported by immunohistochemistry. In three cases the tumour could be removed by radical surgery and none of these patients had a recurrence (median follow-up time: 4 years). The tumour could not be removed completely from one patient with pre-operative chylascos. Six months after diagnosis of LA this patient died of cardiopulmonary failure due to progressive tumour chylascos. Isolation and ligation of the cystic LA's peduncle as well as ligation of lymph channels can prevent recurrences and chylascos.