Cystic lymphangioma of the pancreas

Abstract

Lymphangiomas are benign cystic tumours of lymphatic origin. Most lymphangiomas occur in the neck and axillary region, and <1% occur in the mesentery or retroperitoneum. Lymphangiomas arising from the pancreas are extremely rare, with fewer than 70 published cases. Histologically, they are polycystic, with the cysts separated by thin septa and lined with endothelial cells. Though congenital, it can affect all age groups, and occurs more frequently in children and females. The authors report the case of a 53-year-old man, in whom a polycystic mass, 28 mm x 25 mm in size, was incidentally discovered by computed tomography in the tail of pancreas, during preoperative examination for a large asymptomatic epigastrocele. At surgery, a well circumscribed polycystic tumour was completely excised, with preservation of the pancreatic duct and the spleen. Histology confirmed a microcystic lymphangioma of the pancreas. Immunohistochemistry showed cystic endothelial cells reactivity to factor VIII-RA (++), CD31 (+++) and CD34 (±). The postoperative recovery was uneventful and the patient remained symptom free for two years. Although extremely rare, it is always a challenge to differentiate lymphangioma of the pancreas from other possible cystic – like neoplasms and should be taken into consideration.