Retroperitoneal lymphangioma in a tertiary care hospital of North India

Abstract

Less than 200 cases of retroperitoneal lymphangiomas (RL), a rare type of benign cystic tumour of the lymphatic system that makes about 1% of all lymphangiomas, have been documented to date. Their rarity makes preoperative diagnosis challenging. 95% of lymphangiomas are found in the head, neck, and axilla where they are most frequently found. We are presenting a 30 years old woman, who presented to outpatient department (OPD) of general surgery with symptoms of recurrent left lumbar pain, radiating towards inguinal region for one month. The contrast computed tomography (CT) scan of abdomen and pelvis defined a large thin walled homogenous non enhancing hypodense cystic lesion in left side of abdomen in retroperitoneal location from left lobe of liver till pelvis on left side craniocaudally. The patient was planned for exploratory laparotomy. Midline laparotomy was performed. A cystic lymph-filled tumour was detected in the abdominal cavity adjacent to the bowel loops. Histologically the mass contained variable-sized cystic spaces lined by flattened endothelium consistent with lymphatic vessels, which on immunostaining were positive for CD31 and negative for Pan CK. The patient was discharged satisfactorily. In conclusion, retroperitoneal lymphangioma presents vaguely as abdominal pain or mass and grown to large sizes without causing any significant symptom. Tumour markers for the excised tissue are important for diagnosis. Laparotomy or laparoscopy are the two surgical excision methods used to treat retroperitoneal cystic lymphangioma and it responds well to excision.